Mateo Porres-Aguilar

Portopulmonary hypertension and hepatopulmonary syndrome: a clinician-oriented overview

Liver disease and portal hypertension can be associated with pulmonary vascular complications, including portopulmonary hypertension (POPH), characterised by an elevated mean pulmonary artery pressure secondary to an increased pulmonary vascular resistance, and hepatopulmonary syndrome (HPS), characterised by hypoxaemia due to pulmonary vasodilatation and shunting. Although clear diagnostic guidelines exist for both conditions on the basis of echocardiography, right heart catheterisation and arterial blood gases, there is considerable variation between centres regarding diagnosis and management of these conditions. Awareness of evaluation and management algorithms for POPH and HPS are critical for optimisation of outcomes in patients with these conditions. Key aspects of management of POPH and HPS include identification of patients likely to benefit from liver transplantation (LTx) and management before and after LTx. Although both disorders may improve after LTx, severe forms of POPH represent a contraindication to LTx. Novel approaches to the treatment of POPH and HPS offer new management options that may expand the pool of transplantable patients and improve overall outcomes.

DRESS syndrome following ciprofloxacin exposure: An unusual association

Patient: Female, 24 Final Diagnosis: DRESS syndrome Symptoms: Fever • rash • facial and body swelling • muscular pain • diarrhea Medication: Ciprofloxacin Clinical Procedure: — Specialty: Internal Medicine • Hematology Objective: Unusual or unexpected effect of treatment Background: Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) is a rare, potentially life-threatening drug reaction with a mean latency period of 3.9 weeks. It has been described in association with many medications; yet, Ciprofloxacin was reported once in literature to be the offending drug. We are presenting a rare case of Ciprofloxacin-induced DRESS syndrome with symptom onset 2 days after exposure. Case Report: A 24-year-old female presented with symptoms suggestive of UTI and was prescribed Ciprofloxacin. Two days later she started to complain of fever, diffuse rash, swelling of her face, arms and flanks, watery diarrhea and muscular pain. Five days after symptom onset she presented to our institution with WBC count 38,810 cells/μL and absolute Eosinophil count 17,080 cell/μL. Peripheral blood smear showed immature Eosinophilic myelocytes and metamyelocytes. Skin biopsy showed superficial perivascular lymphocytic infiltration with dermal edema. Further workup was negative for parasitic infestations, HIV and viral hepatitis, allergic diseases, hematologic and non-hematologic malignancies, vasculitides and autoimmune processes. Ciprofloxacin was discontinued upon admission and the patient started to improve quickly. Patient was followed in clinic after 3 weeks and was completely asymptomatic with WBC count 7,250 cell/μL and absolute Eosinophil count 2,900 cell/μL. Conclusions: DRESS syndrome is a possible complication of Ciprofloxacin treatment that clinicians should consider. Shorter latency period might be a unique feature of Fluoroquinolone-induced DRESS syndrome. According to RegiSCAR scoring system, our case is categorized as (probable) with a score of (4). In fact, the vast majority of reported cases are classified as (probable/definite).

Portopulmonary hypertension: An update

Portopulmonary hypertension represents a serious lung vascular disorder, defined as the presence of pulmonary arterial hypertension that is associated with portal hypertension, with or without the presence of significant liver disease. Transthoracic echocardiography represents the single best initial tool for the diagnostic evaluation in portopulmonary hypertension, and right heart catheterization remains the gold standard for definitive diagnosis. Despite the lack of randomized controlled trials in portopulmonary hypertension, some therapies have demonstrated improvements in cardiopulmonary haemodynamics and right ventricular function as described in case reports and case series. Specialists should be able to recognize indications and contraindications for liver transplantation in the setting of portopulmonary hypertension, and this review focuses on the appropriate diagnostic approach and current advances in medical therapies. Recognition of patients eligible for liver transplantation is needed to improve quality of life and survival.

Chronic Thromboembolic Pulmonary Hypertension as an Uncommon Presentation of Primary Antiphospholipid Syndrome

Antiphospholipid syndrome is an autoimmune disease characterized pathophysiologically by the presence of antiphospholipid antibodies and > or =1 clinical manifestation, the most common being venous or arterial thrombosis. We describe the case of a 40-year-old male with unexplained severe pulmonary arterial hypertension with a seven-day history of progressive shortness of breath, hemoptysis, chest discomfort and bilateral pedal edema. Electrocardiographic, echocardiographic and imaging studies showed changes consistent with chronic thromboembolic pulmonary hypertension. Further work-up showed positive anticardiolipin antibodies and lupus anticoagulant with negative features for lupus with negative primary thrombophilic studies as well. The patient was managed adequately with oral anticoagulation with improvement of his clinical status and referred to a tertiary care center to be screened for pulmonary thromboendarterectomy. For patients meeting surgical selection criteria, pulmonary thromboendarterectomy has demonstrated positive outcomes with respect to survival, functionality and quality of life. We discuss the pathophysiology and treatment as well as novel therapies in nonsurgical candidates with chronic thromboembolic pulmonary hypertension in the setting of primary antiphospholipid syndrome.

Pulmonary vascular complications in portal hypertension and liver disease: A concise review

Chronic liver disease and/or portal hypertension may be associated with one of the two pulmonary vascular complications: portopulmonary hypertension and hepatopulmonary syndrome. These pulmonary vascular disorders are notoriously underdiagnosed; however, they have a substantial negative impact on survival and require special attention in order to understand their diagnostic approach and to select the best therapeutic options. Portopulmonary hypertension results from excessive vasoconstriction, vascular remodeling, and proliferative and thrombotic events within the pulmonary circulation that lead to progressive right ventricular failure and ultimately to death. On the other hand, abnormal intrapulmonary vascular dilations, profound hypoxemia, and a wide alveolar-arterial gradient are the hallmarks of the hepatopulmonary syndrome, resulting in difficult-to-treat hypoxemia. The aim of this review is to summarize the latest pathophysiologic concepts, diagnostic approach, therapy, and prognosis of portopulmonary hypertension and hepatopulmonary syndrome, as well as to discuss the role of liver transplantation as a definitive therapy in selected patients with these conditions.

A 38-Year-Old Pregnant Woman with Hemoptysis and Acute Renal Failure

and dysmorphic red blood cell casts. A quantitative serum pregnancy test was positive. Bronchoalveolar lavage retrieved blood-tinged fluid, numerous red blood cells and hemosiderin-laden macrophages but few neutrophils. Microbiology studies for bacteria, mycobacteria and fungi in the bronchoalveolar lavage fluid were negative. Perinuclear antineutrophil cytoplasmic antibodies (P-ANCA) with specificity for myeloperoxidase (MPO) were positive (5.5 U/ml, normal ! 0.4). Serologies for antiglomerular basement membrane, antinuclear antibodies, C-ANCA, antiproteinase 3 (PR3-ANCA), antinuclear antibodies, antiphospholipid panel, and HIV were negative.

Transthoracic echocardiography screening for the detection of portopulmonary hypertension: A work in progress

Mateo Porres-Aguilar, Andres Duarte-Rojo, and Michael J. Krowka Division of Hospital Medicine, Department of Internal Medicine, Paul L. Foster School of Medicine, Texas Tech University Health Sciences Center, El Paso, TX; Division of Gastroenterology and Hepatology, University of Arkansas for Medical Sciences, Little Rock, AR; and Division of Pulmonary and Critical Care Medicine, von Liebig Transplant Center, Mayo Clinic, Rochester, MN

Diffuse alveolar hemorrhage as an initial presentation of systemic lupus erythematosus.

Diffuse alveolar hemorrhage is a rare but fatal complication in patients with systemic lupus erythematosus. It has rarely been reported to occur as the initial presentation in lupus patients. We report a 55-year-old female Jehovahs witness who presented with diffuse alveolar hemorrhage as the initial manifestation of systemic lupus erythematosus. She responded favorably to early intravenous pulse methylprednisolone and cyclophosphamide along with hemotherapy she accepted once over the course of her hospital stay. Initially, there was clinical improvement, but relapsing of her alveolar hemorrhage 15 days later. It is important to keep in mind that lupus patients can present initially with this lethal pulmonary complication. Early identification and initiation of therapy are crucial in order to affect survival of these patients.

Cardiopulmonary hemodynamic clues for pulmonary vein stenosis diagnosis

Pulmonary vein stenosis (PVS) post radiofrequency ablation for chronic atrial fibrillation poses a diagnostic challenge for the clinician. PVS presents with nonspecific symptoms, signs and radiographic features, and may be associated with significant pulmonary vascular involvement. Interestingly, others have described variation of the pulmonary artery wedge pressure between sites of the lung as a clue to pulmonary veno-occlusive disorders. We report, to the best of our knowledge, the first case that describes the regional loss of V waves while recording the mean pulmonary artery wedge pressure (mPawp) as well as the difference in pulmonary artery wedge pressure gradients as the main diagnostic clues for PVS.

Acute nonrheumatic streptococcal myocarditis resembling ST-elevation acute myocardial infarction in a young patient

Acute myocarditis can be induced by various concomitant disease processes including infections. Most of these cases are viral in origin; however, bacterial infections are also implicated to a lesser degree. Group A streptococcus is a frequent culprit in bacterial-induced myocarditis. Its diagnosis is suspected by the presence of signs and symptoms of rheumatic fever as established by the Jones criteria. The development and refinement of current diagnostic tools has improved our ability to identify specific pathogens. It has been found that group A streptococcus may be responsible for more cases of infection-induced acute myocarditis than previously thought, and often without the clinical features of rheumatic fever. We present the case of a 43-year-old man hospitalized with chest pain that was initially diagnosed as an acute ST-elevation myocardial infarction. Further evaluation confirmed that his chief complaint was due to acute nonrheumatic streptococcal myocarditis.