Episodes Of Edema Research Articles

Œdème angioneurotique héréditaire : à propos de deux cas

We report two cases of hereditary angioedema (HAE) in two members of the same family. Angioedema is caused by a deficiency of C1 inhibitor. It shows itself by recurrent, non-inflammatory and non-pruritic episodes of subcutaneous and/or submucosal edema of extremities and face or by pseudo-occlusive abdominal attacks, spontaneously reversible. The exploration of the classical complement pathway during or subsequent to the crisis, fundamental for the diagnosis, shows a decrease of CH50 and C4 complement. The C3 is normal. Weight filling of C1 inhibitor, collapsed in such cases, signs the diagnosis. The occurrence of angioedema in a mother and her daughter suggests a genetic origin; the hereditary form of the disease is most frequently described in the literature. However, in our case, antinuclear antibodies (ANA) detected at a relatively high titer, in one case, and the association of angioedema with rheumatoid arthritis, in the other case, raises the problem of an acquired associated component on this autoimmune background. Nous rapportons deux cas d’œdème angioneurotique héréditaire (OAH) chez deux membres d’une même famille. L’œdème angioneurotique est dû à un déficit en C1 inhibiteur. Il se manifeste par des épisodes récurrents d’œdèmes sous-cutanés et/ou sous-muqueux, non inflammatoires et non prurigineux, des extrémités et du visage ou par des crises abdominales pseudo-occlusives, spontanément réversibles. L’exploration de la voie classique du complément au cours ou au décours des crises, fondamentale pour le diagnostic, révèle une baisse du CH50 et de la fraction C4 du complément. Le C3 reste normal. Le dosage pondéral du C1 inhibiteur, effondré dans ces cas, signe le diagnostic. La survenue d’un œdème angioneurotique chez une mère et sa fille suggère une origine génétique, la forme héréditaire de la maladie étant la plus fréquemment décrite dans la littérature. Toutefois, dans notre observation, les anticorps antinucléaires (AAN) détectés à un titre relativement élevé dans un cas, et l’association de l’œdème angioneurotique à une polyarthrite rhumatoïde dans l’autre cas, posent le problème d’une composante acquise associée sur ce terrain auto-immun.

A12 Ischemic Heart Disease and Weaning from Mechanical Ventilation, how efficient Esmolol can be when Extubation is unsuccessful?

Background and Goal of Study: Patients with ischemic heart disease are known to tolerate hemodynamic stress poorly. As a consequence of their coronary artery disease, ischemic episodes may develop during, or shortly after tracheal tube removal. We describe a case with coronary artery disease that developed repeated episodes of pulmonary oedema each time extubation was attempted. We describe a case of patient with ischemic heart disease and the difficulties in weaning and extubation. Methods: A 70 year old male was submitted urgently because of an abdominal aneurysm. The patient was hemodynamically stable throughout the intervention that lasted 4 hours and on ICU admission, (Heart rate 100/min, and blood pressure 130/65mmHg). Despite trying twice the patient did not achieve successful extubation, in both instances, after extubation, he presented with a rapid shallow breathing and low arterial oxygen saturation. On auscultation, he presented crepitations in middle zones of both lung fields and the chest X-ray was indicative for pulmonary oedema. A trans-thoracic echo that was performed showed normal systolic ventricular function and diastolic dysfunction with abnormal relaxation, patient was therefore given initially esmolol 500 μg/kg/min (iv.) followed by an infusion of 50 μg/kg/min. Sedation was gradually decreased and infusion rate of esmolol was increased. Patient was extubated successfully under 100 μg/kg/min and was discharged from the ICU two days later. Results: Extubation stress in patients with coronary artery disease cause excessive increases in myocardial oxygen consumption thereby altering the supply/demand ratio. Our patient was experiencing ischemic left ventricular dysfunction during weaning, secondary to catecholamine release due to upper airway stimulation by the tracheal tube. This was manifested as hypertension, tachycardia and episodes of pulmonary oedema, which were rapidly resolved with sedation. Conclusions: Using esmolol, a short/acting beta/blocker, allowed successful extubation of patient who developed repeated episodes of pulmonary oedema each time extubation was attempted.

549 Management of Twenty-Five Pediatric Patients with Hereditary Angioedema (Hae) Undergoing Home Treatment—A Clinical Surveillance Program

BackgroundHereditary angioedema (HAE) is a rare disorder characterized by C1 esterase inhibitor (C1-INH) deficiency. Clinically, HAE is characterized by relapsing episodes of edema at various body sites followed by disease-free intervals of variable duration. Episodes of upper airway obstruction (usually laryngeal edema) are potentially life-threatening and many patients died by asphyxiation in the families. In literature Longhurst et al, Buygun et al and Levi et al showed improvement of quality of life in patients with hereditary angioedema due to home treatment with C1-inhibitor-concentrate.MethodsWe investigated in a cohort study the integration of a clinical surveillance program into home treatment of pediatric patients suffering from hereditary angioedema. Parameters investigated were overall coping of the pediatric patient with home treatment, documentation of efficacy/safety of C1-INH concentrate (Berinertâ P, CSL Behring, Marburg), regular control of laboratory parameters (C1-inhibitor (INH) activity, C1-INH antigen, C4, hepatitis A, -B, -C-, HIV-1/2-, and parvovirus B19 serology) and quality of life parameters (hospitalization, absence from school).ResultsTwenty-five pediatric HAE patients (6 male, 19 female) have so far been investigated. Twenty-one patients suffer from HAE type I, 4 patients from HAE type II. Median age is 13.7 years (range: 2.8–17.4 years), first diagnosis of HAE took place at the median age of 5 years (range: 0.1–15.9 years) and first manifestation of HAE at the median age of 3.9 years (range: 0.3–11.7 years). Plasma C4 complement was reduced in nearly all patients (median: 2.4 mg/dL; range: <1.4–10 mg/dL) except one patient. All patients coped well with home treatment, compliance was excellent, clinical findings during regular medical control remained in the normal range and all parameters confirmed an improved quality of life, e.g., patients had not been hospitalized nor had they been absent from school. There were no adverse drug reactions due to administration of C1-INH concentrate.ConclusionsHome treatment might be also a valuable option for pediatric HAE patients not affecting compliance negatively and providing a significant positive impact on health-related quality of life.

Acute compartment syndrome of upper limb as an unusual complication of dengue hemorrhagic fever

Dear Editor, Dengue fever with its hemorrhagic complications is a common viral illness in India and other south Asian countries. We present a case of dengue hemorrhagic fever associated with capillary leak syndrome presenting as a compartment syndrome involving the right upper limb in the background of iron deficiency, which we believe to be the first such presentation of dengue in medical literature. Our patient, a 40-year-old man, presented with high grade continuous fever, retro-orbital headache, and generalized body ache for 5 days along with scanty gum bleeding for 2 days. He also had red spots and swelling of the right forearm for 2 days, the swelling being gradual in onset, painful, reddish in appearance, and rapidly progressing to restrict movements of the right upper limb. There was no history of trauma, insect bites, rash, drug reaction, or past history suggestive of any coagulation disorder. On examination, the patient was febrile, conscious, oriented, had mild pallor, and active gum bleeding in the oral mucosa. There was a warm, tense, tender, erythematous swelling involving the entire right forearm, wrist, hand, and all fingers, with hypoaesthesia involving all sensory modalities but without any ulceration or discharge. Both active and passive movements at all joints involving the right hand and wrist were severely painfully restricted and resisted. Laboratory tests revealed hemoglobin (Hb) 7.2 g/dL, platelets 15,000/μL and Hematocrit 58%, and erythrocyte sedimentation rate (ESR) of 36 mm in first hour. Abnormal cells or parasites were absent on the peripheral smear. Biochemical tests including liver function test (LFT), kidney function test (KFT), electrolytes and urine routine, and microscopic examination (RE/ME) were all normal. Malarial antigen tests were repeatedly negative. Dengue IgG and IgM were strongly positive and an initial diagnosis of dengue hemorrhagic fever was entertained. Disseminated intravascular coagulation (DIC) was ruled out with a normal serum level of fibrin degradation products (8.2 μg/dL). Platelet transfusion support, fluids, and paracetamol were instituted. Chest X-ray, X-rays of the right arm, forearm, and hand were all normal. Ultrasonic (USG) Doppler of the right forearm documented gross soft tissue swelling with no compromise of the venous or arterial blood flow. Orthopedic consultation concurred with our diagnosis of right forearm compartment syndrome with Volkmann's Ischemic contracture (VIC) grade III. Despite low platelets, high risk surgical decompression of the right forearm was performed after 2 days with blood component support, and extensive muscle swelling discoloration was demonstrated during operation. Large clots were removed from the anterior compartment and the muscles began to regain their natural color immediately following fasciotomy. Post-operative recovery was uneventful over the next 12 days [Figure 1], without any fever, secondary infection, or swelling on any other site of the body. Hb and platelet counts subsequently rose to 8.6 g/dL and 1,10,000/μL, respectively. Secondary closure of right forearm fasciotomy was done at 2 week follow-up. Figure 1 Photograph of the right forearm 3 days after emergency fasciotomy. The edematous muscles of the anterior compartment of the right forearm are visibl As documented in the literature, the cardinal feature of dengue hemorrhagic fever (DHF) results from a transient increase in vascular permeability that causes fluid leakage from the intravascular to the interstitial compartment, leading to hemoconcentration, and circulatory collapse, which contribute to mortality and significant morbidity.[1] Capillary leak syndrome is seen in various situations, sepsis and dengue being the major infectious causes.[2] The mechanisms of capillary leak may involve increased hydrostatic pressure, decreased oncotic pressure, or increased permeability of the capillary endothelium.[3] Among the other infectious causes of capillary leak leading to compartment syndrome, Brucellosis, Hantavirus infection, and sepsis have been mentioned in literature.[4] Idiopathic systemic capillary leak syndrome (SCLS) or Clarkson's disease is another important cause of capillary leakage, where there are recurrent episodes of hypovolemic shock, edema, hemoconcentration, and hypoalbuminemia. Episodes of systemic capillary hyperpermeability due to various cytokines have been implicated in the pathogenesis of this disorder.[5,6] The most common bleeding manifestations of dengue include petechiae, gum bleeding, GI bleeding and hemoptysis although rarer sites are often involved.[7] Even after thorough clinical and radiological evaluation (chest skiagram and abdominal ultrasound), we did not find evidence of capillary leakage at the common sites like pleural cavity, pericardial cavity, and peritoneum. Workers from South India have shown that bleeding in dengue might lead to abdominal compartment syndrome in rare cases.[8] The solitary involvement of the right forearm in this scenario is indeed uncommon, and adds to the clinical interest of this case. In conclusion, internists must be aware of these different forms of dengue complications, where urgent and aggressive interventions may enable physicians to salvage limbs and prevent mortality. We report this case to underline the importance of internal bleeding and capillary leak in dengue, as also to record a clinical scenario as yet unreported in the world literature.

Human Plasma-Derived, Nanofiltered, C1-Inhibitor Concentrate (Cinryze®), a Novel Therapeutic Alternative for the Management of Hereditary Angioedema Resulting from C1-Inhibitor Deficiency

Hereditary angioedema resulting from the deficiency of the C1 inhibitor (HAE-C1-INH) is a rare, but potentially life-threatening disorder characterized by paroxysmal episodes of subcutaneous or submucosal edema. Early diagnosis is essential. Management is aimed at the prompt elimination of full-fledged attacks, as well as at the prevention of edematous episodes. The most straightforward means for therapy is supplementation with the deficient C1-INH protein. Placebo-controlled and open clinical studies have established that nanofiltered, human C1-INH concentrate, Cinryze® (ViroPharma Inc., Exton, PA, USA) (C1-INHCi), administered in 1,000 U doses is an effective and safe remedy for edematous episodes of HAE-C1-INH, regardless of the localization of the attack. Clinical manifestations rapidly improve and then resolve completely following treatment with this medicinal product. Additionally, C1-INHCi is also appropriate for pre-procedural or for routine prophylaxis. The administration of 1,000 U C1-INHCi before the (dental, surgical, or interventional diagnostic) procedure reduced the incidence of edematous episodes compared with placebo, and this reduction proved significant during routine prophylaxis with the administration of this dose every 3–4 days. Relapses did not occur, and repeated dosing had no influence on the efficacy of the preparation. Patients also tolerated treatment with C1-INHCi well. The safety of this preparation was confirmed by the absence of viral transmission as well as by the lack of antibody formation against C1-INH during treatment. Nowadays, C1-INHCi for intravenous use is the only medicinal product indicated both for the prevention and management of edematous attacks.

Recent Advances in Management and Treatment of Hereditary Angioedema

Hereditary angioedema (HAE) is a rare autosomal-dominant disease characterized by recurrent self-limiting episodes of skin and mucosal edema. Morbidity and mortality are significant, and new and pending therapies are now available to reduce the risk associated with the disease. To update the reader on new advances in HAE to improve patient care. We performed a literature search of Ovid, PubMed, and Google to develop this review. Articles that are necessary for the understanding and use of the new therapeutic options for HAE were chosen, and studies of high quality were used to support the use of therapies, and in most cases, results from phase III studies were used. Until recently, therapy for HAE attacks in the United States consisted of symptom relief with narcotics, hydration, and fresh-frozen plasma, which contains active C1 inhibitor. Therapy to prevent HAE attacks has been confined to androgens and, occasionally, antifibrinolytic agents; however, both drug groups have significant adverse effects. The approval of C1-inhibitor concentrate for prevention and acute therapy has improved efficacy and safety. Ecallantide has also been approved for therapy of attacks, and icatibant is expected to be approved in the next few months for attacks. Recombinant C1 inhibitor is presently in phase III studies and should be available for attacks in the near future. In this article we review the changing therapeutic options available for patients in 2011 and beyond.

New treatment options for acute edema attacks caused by hereditary angioedema

New treatment options for acute edema attacks caused by hereditary angioedema (HAE) are reviewed. HAE is characterized by mutations in the C1 inhibitor gene leading to either a reduced expression of C1 inhibitor in the plasma or expression of a functionally impaired C1 inhibitor. HAE is classified into two major types based on the cause of the C1 inhibitor deficiency. Type I HAE is defined by a reduced expression of C1 inhibitor in the plasma, whereas type II HAE is characterized by the expression of a dysfunctional C1 inhibitor protein. Clinical data were reviewed for C1 inhibitor, ecallantide, and icatibant in the treatment of acute edema attacks caused by HAE. C1 inhibitor leads to a faster onset of edema relief and is effective in decreasing the duration of edema. Dosing strategies include fixed dosing and weight-based dosing. Optimal dosing strategies have not been established, but fixed dosing (500-1000 units) or 20 units/kg has been effective in clinical trials and reports. No comparative trials suggest that one strategy is superior to another; however, the approved labeling for acute treatment is based on weight. Ecallantide is also efficacious for treating acute episodes; however, the available evidence is limited to a single published trial. Icatibant has shown variable effects in two trials with placebo and active controls. In patients with HAE, most edema episodes only involve the skin and gastrointestinal tract, though airway obstruction caused by laryngeal angioedema is the most common cause of death. I.V. C1 inhibitor should be considered first-line treatment for acute edema attacks because of its fast onset of action and effectiveness, though it is not clear whether fixed or weight-based dosing is preferred. Ecallantide can be considered as a second-line treatment option.

Clinical management of angioneurotic edema patient post orthognathic surgery

Angioneurotic edema is an acute swelling involving the submucosal or subcutaneous tissues, being most often located in the oral and maxillofacial region, which can result in upper airway obstruction. Its etiology is frequently associated with immunoglobulin-mediated hypersensitivity reactions that elicit a heightened inflammatory response. The objective of this study was to report the case of a patient who developed an episode of angioneurotic edema following combined orthognathic surgery. Pharmacological and nonpharmacological interventions used in the treatment of this important clinical condition are described and discussed.

A large heterozygous deletion including the entire C1 inhibitor gene in a sporadic case of hereditary angio-oedema

C1 inhibitor (C1-INH) deficiency [hereditary or acquired angio-oedema (HAE or AAE)] is characterized by recurring episodes of subcutaneous or submucosal oedema. Many different mutations in the C1-INH gene have been identified as a cause of HAE. We investigated the molecular basis of the disease in a Japanese woman with sporadic HAE. Direct sequencing of genomic DNA revealed no point mutation in the C1-INH gene. Quantitative real-time PCR showed that the copy number of the C1-INH gene in the patient was half that of a healthy control. Furthermore, we identified a 650-kbp deletion on the chromosome, which included the C1-INH gene. We evaluated the correlation between the patient's attacks and her coagulation activity. The levels of D-dimer were high during the angio-oedema attacks, and often exceeded the normal range even during remission, thus the level of D-dimer reflected the activity of HAE in this patient.

Advances in the therapy of hereditary angioedema

Hereditary angioedema is a rare autosomal dominantly inherited disease characterized by recurrent episodes of subcutaneous and mucosal edema due to the deficiency of plasma functional C1 esterase inhibitor.Acute attacks of laryngeal swelling are often associated with a substantial risk of death.In the past,hereditary angioedema was treated with anti-histamine drugs and glucocorticoids,but the outcome was unsatisfactory,and mortality was reported as high as 30%.With further insights into the pathogenesis of hereditary angioedema,new drugs have emerged and provided clinicians and patients with more choices.The authors summarize the pathogenesis and clinical manifestations of the entity with focus on the progress in its treatment. Key words: Angioneurotic edema; Heredity; C1 esterase inhibitors; Therapy

Anemia infecciosa equina

A Anemia Infecciosa Equina (AIE) é uma doença viral crônica, causada por um vírus da família Retroviridae, gênero Lentivirus, limitada a equinos, asininos e muares, caracterizada por episódios periódicos de febre, anemia hemolítica, icterícia, depressão, edema e perda de peso. A AIE gera embargos ao trânsito de equídeos, além de interferir nos eventos esportivos equestres, assumindo assim uma relevância econômica considerável. O agente é transmitido primariamente por picadas de tabanídeos (Tabanus sp.) e moscas dos estábulos (Stomoxys calcitrans) sendo estes apenas vetores mecânicos. Os principais reservatórios da enfermidade são os portadores inaparentes do vírus, principalmente em tropas que não sofrem monitoramento sorológico periódico. A transmissão é mais comum nas épocas mais quentes do ano e em regiões úmidas e pantanosas. A prova da imunodifusão em gel de Agar (IDGA) é considerada o teste padrão-ouro. No Brasil, os laboratórios e técnicos interessados em realizar o diagnóstico devem ser cadastrados no Ministério da Agricultura. As medidas de controle para limitar a disseminação do vírus se baseiam principalmente em testes sorológicos de rotina e na remoção dos animais reagentes do plantel, além da restrição ao deslocamento de animais, do teste dos novos animais a serem introduzidos nas tropas, do controle da população de vetores e do não compartilhamento de seringas, agulhas e outros utensílios que possam ser veículo de células infectadas. No Brasil, os animais positivos no teste de IDGA devem ser sacrificados, conforme estabelecido pelo Programa Nacional de Sanidade dos Equídeos do Ministério da Agricultura. O estudo dos aspectos biológicos, epidemiológicos e profiláticos da doença se apresenta fundamental para o controle desta enfermidade e, consequentemente para o sucesso da equinocultura de qualquer país ou região onde a mesma se faz presente.

A single nucleotide deletion at the C1 inhibitor gene as the cause of hereditary angioedema: insights from a Brazilian family

Hereditary angioedema is an autosomal dominant disease characterized by episodes of subcutaneous and submucosal edema. It is caused by deficiency of the C1 inhibitor protein, leading to elevated levels of bradykinin. More than 200 mutations in C1 inhibitor gene have been reported. The aim of this study was to analyze clinical features of a large family with an index case of hereditary angioedema and to determine the disease-causing mutation in this family. Family pedigree was constructed with 275 individuals distributed in five generations. One hundred and sixty-five subjects were interviewed and investigated for mutation at the C1 inhibitor gene. Subjects reporting a history of recurrent episodes of angioedema and/or abdominal pain attacks underwent evaluation for hereditary angioedema. We have identified a novel mutation at the C1 inhibitor gene, c.351delC, which is a single-nucleotide deletion of a cytosine on exon 3, resulting in frameshift with premature stop codon. Sequencing analysis of the hypothetical truncated C1 inhibitor protein allowed us to conclude that, if transcription occurs, this protein has no biological activity. Twenty-eight members of the family fulfilled diagnostic criteria for hereditary angioedema and all of them presented the c.351delC mutation. Variation in clinical presentation and severity of disease was observed among these patients. One hundred and thirty-seven subjects without hereditary angioedema did not have the c.351delC mutation. The present study provides definitive evidence to link a novel genetic mutation to the development of hereditary angioedema in patients from a Brazilian family.

Recurrent pulmonary edema caused by chronic left main coronary artery occlusion

An 80-year-old patient was referred to our institution after three recurrent episodes of pulmonary edema, and the last one 20 days prior to the outpatient visit. The patient complained of persistent class III dyspnea, but denied chest pain. He was currently receiving aspirin, angiotensin-converting enzyme inhibitors, nitrates, amlodipine and furosemide. The past medical history included prior myocardial infarction 15 years before presentation and hypertension. The last invasive angiogram was performed 13 years before and documented an occluded left anterior descending artery and anterior wall akinesia. The …

Impaired Left Ventricular Stroke Volume Reserve During Clinical Dobutamine Stress Predicts Future Episodes of Pulmonary Edema

The purpose of this study was to determine whether dobutamine-induced abnormal stress changes in left ventricular stroke volume (LVSV) and aortic stiffness predict future pulmonary edema. Increased aortic stiffness that decreases LVSV during adrenergic stress may serve as a marker for future pulmonary edema (PE). We measured LVSV, ventriculovascular stiffness (pulse pressure/LVSV(index)), and aortic distensibility at rest and during intravenous dobutamine administration using cardiovascular magnetic resonance. Personnel blinded to dobutamine cardiovascular magnetic resonance followed participants longitudinally over time to identify those admitted to the hospital with PE. Data for 44 participants who had a hospital admission for PE were compared with data for 72 participants of similar age, sex, and resting left ventricular ejection fraction who remained PE free. Expressed as median and interquartile range, participants with and without PE exhibited a decreased stress/rest LVSV ratio (0.9 [range 0.7 to 1.1] vs. 1.0 [range 0.9 to 1.2], respectively; p = 0.002), an increased ventriculovascular stiffness stress/rest ratio (1.4 [range 1.0 to 1.6] vs. 1.0 [range 0.8 to 1.3], respectively; p ≤ 0.001); and a decreased stress-induced measure of aortic distensibility (0.8 mm Hg(-3) [range 0.3 to 1.3 mm Hg(-3)] vs. 1.6 mm Hg(-3) [range 1.2 to 3.2 mm Hg(-3)], respectively; p = 0.002). After accounting for age, sex, left ventricular ejection fraction, risk factors for PE, and the presence of dobutamine-induced ischemia, LVSV reserve and the stress/rest ventriculovascular stiffness ratio still differed (p < 0.008 for both) in those with and without PE. In patients without inducible ischemia during dobutamine stress testing in whom one might otherwise assume a favorable prognosis, the failure to increase LVSV or an increase in ventriculovascular stiffness indicates patients at risk of subsequent PE.

Angioedema triggered by pulp extirpation—a case report

C1-esterase inhibitor deficiency results in episodes of non-allergic edema of parts of the body. Edema of the face may be triggered by dental therapy. We report a case of C1-esterase inhibitor deficiency which was detected in a 42-year-old woman. The patient was completely unaware that she had this disorder or of any related family history, and the patient developed an intense facial angioedema after pulp extirpation of lower premolar tooth. In this case, the diagnosis of angioedema due to C1-esterase inhibitor deficiency was established at a later stage. The differing causes of C1-esterase inhibitor deficiency are briefly discussed and the treatment modalities outlined.

Successful C1 inhibitor short-term prophylaxis during redo mitral valve replacement in a patient with hereditary angioedema

Hereditary angioedema is characterized by sudden episodes of nonpitting edema that cause discomfort and pain. Typically the extremities, genitalia, trunk, gastrointestinal tract, face, and larynx are affected by attacks of swelling. Laryngeal swelling carries significant risk for asphyxiation. The disease results from mutations in the C1 esterase inhibitor gene that cause C1 esterase inhibitor deficiency. Attacks of hereditary angioedema result from contact, complement, and fibrinolytic plasma cascade activation, where C1 esterase inhibitor irreversibly binds substrates. Patients with hereditary angioedema cannot replenish C1 esterase inhibitor levels on pace with its binding. When C1 esterase inhibitor is depleted in these patients, vasoactive plasma cascade products cause swelling attacks. Trauma is a known trigger for hereditary angioedema attacks, and patients have been denied surgical procedures because of this risk. However, uncomplicated surgeries have been reported. Appropriate prophylaxis can reduce peri-operative morbidity in these patients, despite proteolytic cascade and complement activation during surgical trauma. We report a case of successful short-term prophylaxis with C1 esterase inhibitor in a 51-year-old man with hereditary angioedema who underwent redo mitral valve reconstructive surgery.

Percutaneous mitral valve annuloplasty for functional mitral regurgitation: acute results of the first patient treated with the Viacor permanent device and future perspectives

There is a need to develop less invasive techniques to manage moderate or severe functional mitral regurgitation in patients at high surgical risk. We report the acute results of the first patient treated with the permanent Viacor percutaneous transvenous mitral annuloplasty (PTMA) device in North America, introduce the PTOLEMY-2 protocol, and briefly discuss the current status of transvenous mitral valve techniques. After several episodes of pulmonary edema, an 87-year-old woman was referred for hemodynamic evaluation. Angiography revealed normal coronary arteries and severe mitral regurgitation. Baseline echocardiography showed severe (4+) functional mitral regurgitation. The coronary sinus was cannulated with a 9.5-Fr introducer from a left subclavian approach. After distal positioning of a coronary wire, the 7-Fr PTMA Viacor catheter was advanced to the anterior interventricular vein. Two 130 g/cm rods were then inserted resulting in an acute and dramatic reduction in mitral regurgitation as assessed by continuous transoesophageal echocardiography and which was associated with a sudden rise in arterial blood pressure. The next day, transthoracic echocardiogram showed a significant reduction in effective regurgitant orifice area (EROA) from 41 to 10 mm(2). The patient was discharged home the day following the procedure without complication. In accordance with the PTOLEMY-2 protocol, she will undergo 3-D transthoracic echocardiograms, quality of life assessments, and 6-min walk tests at regular intervals for the next 5 years. PTMA is a promising technique for the treatment of severe mitral regurgitation in selected patients. Further ongoing research will determine the predictors of success and long-term safety and performance of this technique.

Clinical management of angioneurotic oedema patient post-orthognathic surgery

Angioneurotic oedema is an acute swelling involving the submucosal or subcutaneous tissues; it is most often located in the oral and maxillofacial region, which can result in upper airway obstruction. Its aetiology is frequently associated with immunoglobulin-mediated hypersensitivity reactions that elicit a heightened inflammatory response. The objective of this study was to report the case of a patient who developed an episode of angioneurotic oedema following combined orthognathic surgery. Pharmacological and nonpharmacological interventions used in the treatment of this important clinical condition are described and discussed.

Rheumatic mitral valve disease in pregnancy

A 32-year-old Nepalese woman with known rheumatic mitral valve disease presented 17 weeks into her first pregnancy with exertional breathlessness and wheezing, leading to expectoration of foamy pink liquid. Her history included percutaneous balloon mitral valvotomy at the age of 25 years, after which she had remained asymptomatic and fully active. She had three episodes of acute pulmonary oedema and was transferred to a tertiary centre for further management. An echocardiogram suggested severe mitral stenosis (mitral valve area 1.0 cm2) and pulmonary hypertension (mean pulmonary artery pressure 87 mmHg). She had further episodes of pulmonary oedema despite maximal medical therapy with diuretics, cardioselective beta blockers (dose limited because of systolic hypotension), digoxin, nitrates and anticoagulation. At 21 weeks' gestation, an emergency St Jude metallic valve was implanted. Unfortunately, intrauterine death occurred 2 days postoperatively, likely caused by pre-eclampsia or haemolysis, elevated liver enzymes and low platelets (HELLP) syndrome. The patient recovered well and continues on warfarin and a small dose of beta blocker.

Human C1-Esterase Inhibitor Concentrate (Berinert®)

Human C1-esterase inhibitor concentrate (C1-INH concentrate) is derived from human plasma and is indicated for the treatment of acute episodes of hereditary angioedema. Intravenous C1-INH concentrate provided rapid symptom relief in patients with acute abdominal or facial episodes associated with type I or II hereditary angioedema, according to the results of the randomized, double-blind, multicenter, placebo-controlled IMPACT-1 trial. The median time to the onset of symptom relief was significantly shorter with C1-INH concentrate 20 U/kg than with placebo (0.5 vs 1.5 hours). A subsequent noncomparative extension trial (IMPACT-2) showed the ongoing efficacy of 'on-demand' treatment with C1-INH concentrate; overall, the onset of symptom relief occurred in a median 30 minutes. Several additional studies demonstrated the efficacy of intravenous C1-INH concentrate in patients with laryngeal edema, skin swellings, or abdominal episodes associated with type I or II hereditary angioedema. Self-administered home therapy with intravenous C1-INH concentrate significantly improved health-related quality of life in patients with hereditary angioedema. Dermatology Life Quality Index and Short Form 36-item Health Survey scores were significantly improved from baseline by self-administered home therapy with C1-INH concentrate. Intravenous C1-INH concentrate was well tolerated in patients with hereditary angioedema, with no confirmed cases of viral transmission.