Q fever 1985-1998. Clinical and epidemiologic features of 1,383 infections

In order to describe the clinical features and the epidemiologic findings of 1,383 patients hospitalized in France for acute or chronic Q fever, we conducted a retrospective analysis based on 74,702 sera tested in our diagnostic center, National Reference Center and World Health Organization Collaborative Center for Rickettsial Diseases. The physicians in charge of all patients with evidence of acute Q fever (seroconversion and/or presence of IgM) or chronic Q fever (prolonged disease and/or IgG antibody titer to phase I of Coxiella burnetii > or = 800) were asked to complete a questionnaire, which was computerized. A total of 1,070 cases of acute Q fever was recorded. Males were more frequently diagnosed, and most cases were identified in the spring. Cases were observed more frequently in patients between the ages of 30 and 69 years. We classified patients according to the different clinical forms of acute Q fever, hepatitis (40%), pneumonia and hepatitis (20%), pneumonia (17%), isolated fever (17%), meningoencephalitis (1%), myocarditis (1%), pericarditis (1%), and meningitis (0.7%). We showed for the first time, to our knowledge, that different clinical forms of acute Q fever are associated with significantly different patient status. Hepatitis occurred in younger patients, pneumonia in older and more immunocompromised patients, and isolated fever was more common in female patients. Risk factors were not specifically associated with a clinical form except meningoencephalitis and contact with animals. The prognosis was usually good except for those with myocarditis or meningoencephalitis as 13 patients died who were significantly older than others. For chronic Q fever, antibody titers to C. burnetii phase I above 800 and IgA above 50 were predictive in 94% of cases. Among 313 patients with chronic Q fever, 259 had endocarditis, mainly patients with previous valvulopathy; 25 had an infection of vascular aneurysm or prosthesis. Patients with endocarditis or vascular infection were more frequently immunocompromised and older than those with acute Q fever. Fifteen women were infected during pregnancy; they were significantly more exposed to animals and gave birth to only 5 babies, only 2 with a normal birth weight. More rare manifestations observed were chronic hepatitis (8 cases), osteoarticular infection (7 cases), and chronic pericarditis (3 cases). Nineteen patients were observed who experienced first a documented acute infection, then, due to underlying conditions, a chronic infection. To our knowledge, we report the largest series of Q fever to date. Our results indicate that Q fever is a protean disease, grossly underestimated, with some of the clinical manifestations being only recently reported, such as Q fever during pregnancy, chronic vascular infection, osteomyelitis, pericarditis, and myocarditis. Our data confirm that chronic Q fever is mainly determined by host factors and demonstrate for the first time that host factors may also play a role in the clinical expression of acute Q fever.     

Use of gallium 67 scintigraphy to differentiate acute myocarditis from acute myocardial infarction

This study was designed to evaluate the role and effectiveness of gallium 67 imaging in the diagnosis of acute myocarditis that mimics acute myocardial infarction. Of 315 consecutive acute myocardial infarction patients admitted to our institution over a 4-year period, 5 (2 men, 3 women) were suspected of having acute myocarditis. These 5 patients ranged in age from 23 to 69 years (median, 32 yr). All had experienced diarrhea or flu-like symptoms within the preceding 4 weeks, and each presented with signs, symptoms, and electrocardiographic findings consistent with acute myocardial infarction. Echocardiography revealed decreased left ventricular systolic function. Gallium 67 myocardial scintigraphy was performed in 4 patients, 72 hours after intravenous injection of 9 mCi of gallium citrate Ga 67, and sooner than that in one. In all 5 patients, the results were positive, consistent with a diagnosis of acute myocarditis. One patient died of progressive heart failure 4 days after admission. Within 1 month of beginning medical therapy, the 4 surviving patients experienced resolution of abnormal ventricular function and symptoms. During a median follow-up period of 64 months, no cardiovascular events were observed, and the prognoses were deemed excellent. We suggest that gallium 67 scintigraphy is a useful method by which to identify acute myocarditis in patients in whom the condition is suspected. To our knowledge, this is the 1st report of the use of gallium 67 myocardial scanning to differentiate acute myocarditis from acute myocardial infarction.     

Clinical manifestations of influenza a myocarditis during the influenza epidemic of winter 1998-1999.

OBJECTIVES: The clinical features of myocarditis that developed during the influenza epidemic of winter 1998-1999 were investigated to emphasize the need for medical attention to this disease. METHODS: Nine patients were treated under diagnoses of acute myocarditis during the winter of 1998-1999. Five (two males and three females, mean age 52 +/- 18 years) were examined with myocarditis associated with influenza A. The diagnosis of influenza A myocarditis was based on electrocardiographic and echocardiographic abnormalities, increased creatine kinase levels and at least a four-fold increase in influenza A virus titers using paired sera. RESULTS: All patients had preceding flu-like symptoms and fever. Cardiac involvement developed between 4 and 7 days after the onset of influenza symptoms. Dyspnea progressively worsened in three patients, one went into shock and one had persistent fever, cough and mild dyspnea without apparent cardiac symptoms. Three patients had ST elevation associated with Q waves and one had complete left bundle branch block. The creatine kinase levels were abnormally increased and global wall motion of the left ventricle on echocardiography was decreased in all patients. Two patients had diagnoses of fulminant myocarditis. One patient died of pneumonia following cerebral infarction, but the left ventricular dysfunction normalized in the remaining four patients. CONCLUSIONS: Cardiac involvement occurred between 4 and 7 days after the onset of influenza symptoms, and worsening dyspnea was the most common symptom. Electrocardiography, echocardiography and creatine kinase levels should be checked to determine the potential for cardiac involvement when patients present with suspected influenza associated with worsening dyspnea or prolonged weakness. Increasing the awareness of influenza myocarditis may help in the earlier identification and treatment of this disease during influenza epidemics.     

Risks factors and prevention of Q fever endocarditis.

Coxiella burnetii causes acute and chronic Q fever. To evaluate the risk factors of development of chronic endocarditis following Q fever and to assess the best preventive therapy, a retrospective study of patients diagnosed as having Q fever during 1985-2000 was conducted. Twelve patients with acute Q fever who developed endocarditis and 102 patients with Q fever endocarditis were included in the study. When compared to 200 control patients with acute Q fever, preexisting valvular disease (P<10(-7)), especially a prosthetic valve (P=.01), were encountered more often among patients with endocarditis. Among patients with valvular defects, we estimate the risk of developing endocarditis to be 39%. A combination of doxycycline plus hydroxychloroquine was better at preventing the development of endocarditis than doxycycline alone (P=.009). Our results should encourage physicians to detect valvular lesions in patients with acute Q fever and to search for acute Q fever in patients with a valvulopathy and unexplained fever. A proper treatment for such patients and a scheduled follow-up should reduce the risk of developing endocarditis.     

Endomyocardial biopsy in childhood]

The experience with endomyocardial biopsy in pediatric age is still limited. From February 1986 to August 1990, 144 right ventricle endomyocardial biopsies were performed in 84 patients (age range 33 days--14 years, median age 31 months, weight range 3--57 kgs). Clinical diagnosis was: dilated cardiomyopathy in 50 patients; graft reject in 19; hypertrophic cardiomyopathy in 4; restrictive cardiomyopathy in 5; heart tumor in 3; ventricular arrhythmia in 3. The bioptome was introduced directly, without the use of a long sheath. There were no major complications; 2 patients experienced complete transient atrioventricular block and in 1 case right ventricular perforation occurred. In 11/45 patients (27%) with the clinical diagnosis of dilated cardiomyopathy and available myocardial specimens, acute myocarditis was diagnosed. In 47/65 procedures in the transplanted patients, a moderate to severe rejection was diagnosed. In the remaining patients, endomyocardial biopsy did not help the clinical diagnosis. We conclude that the right ventricular endomyocardial biopsy is a safe procedure in pediatric age; its utility is mostly limited to the diagnosis of acute myocarditis and graft reject after cardiac transplantation.     

Coronavirus-induced myocarditis: A meta-summary of cases

BackgroundMyocarditis caused by SARS-CoV-2 infection was proposed to account for a proportion of cardiac injury in patients with COVID-19. However, reports of coronavirus-induced myocarditis were scarce. The aim of this review was to summarise the published cases of myocarditis and describe their presentations, diagnostic processes, clinical characteristics and outcomes.MethodsA literature search of MEDLINE, EMBASE, Scopus, Web of Science, CENTRAL and OpenGrey on was performed on 3 June 2020. Studies of myocarditis in patients with COVID-19 were included, and those only reporting cardiac injury or heart failure were excluded. Cases were “confirmed” myocarditis if diagnosed on cardiac magnetic resonance imaging (CMR) or histopathology. Those without were grouped as “possible” myocarditis.ResultsA total of 31 studies on 51 patients were included; 12 cases were confirmed myocarditis while 39 had possible myocarditis. The median age was 55 and 69% were male. The most common presenting symptoms were fever, shortness of breath, cough and chest pain. Electrocardiogram changes included non-specific ST-segment and T-wave changes and ventricular tachycardia. Most patients had elevated cardiac and inflammatory biomarkers. Left ventricular dysfunction and hypokinesis was common. CMR established the diagnosis in 10 patients, with features of cardiac oedema and cardiac injury. Five patients had histopathological examination. Some cases required mechanical ventilation and extracoporeal membrane oxygenation, and 30% of patients recovered but 27% died.ConclusionsCOVID-19 myocarditis was associated with ECG, cardiac biomarker and echocardiographic changes, and the manifestation could be severe leading to mortality. Endomyocardial biopsy was not available in most cases but CMR was valuable.     

Outcomes for children with acute myocarditis

The optimum treatment for myocarditis in children is unknown. We present outcomes for this disease as seen in a large series of children. Thus, we identified all children seen with myocarditis at Children's Hospital of Pittsburgh since 1985, including only those with biopsy-proven myocarditis, or cardiac dysfunction and proof of concomitant cardiotropic viral infection. Outcomes were defined as complete recovery, incomplete recovery, and death or transplantation. We identified 41 patients, 37 proven by histology, and 4 patients who were too unstable for biopsy but had proof of viral infection. Of the group, 27 (66%) made a complete recovery, 4 (10%) had incomplete recovery, and 10 (24%) either died (5) or underwent transplantation (5). The median time to death or transplantation was 8.4 months, with a range from 1 day to 49 months. Steroids had been administered to 16 patients, of whom 10 made a complete recovery, 2 an incomplete recovery, 2 died, and 2 were transplanted. Intravenous immune globulin was given in isolation to one patient, who made a complete recovery, and to 18 in combination with steroids, of whom 12 made a complete recovery, 2 an incomplete recovery, 2 died, and 2 were transplanted. The remaining 6 patients received neither steroids nor intravenous immune globulin, and of these, 4 made a complete recovery, 1 was transplanted, and 1 died. Freedom from death or transplantation was 81% at 1 year, and 74% at 5 years, with no difference between the modes of treatments. The median time to recovery of function was also comparable between the groups. Thus, in our patients, treatment with intravenous immune globulin appeared to confer no advantage to steroid therapy alone. These data emphasise the need for randomised trials to assess the efficacy of current treatments, as well as that of new therapies.     

From discrete dilated cardiomyopathy to successful cardiac transplantation in congenital disorders of glycosylation due to dolichol kinase deficiency (DK1-CDG)

Congenital disorders of glycosylation are a growing group of inborn errors of protein glycosylation. Cardiac involvement is frequently observed in the most common form, PMM2-CDG, especially hypertrophic cardiomyopathy. Dilated cardiomyopathy, however, has been only observed in a few CDG subtypes, usually with a lethal outcome. We report on cardiac pathology in nine patients from three unrelated Israeli families, diagnosed with dolichol kinase deficiency, due to novel, homozygous DK1 gene mutations. The cardiac symptoms varied from discrete, mild dilation to overt heart failure with death. Two children died unexpectedly with acute symptoms of heart failure before the diagnosis of DK1-CDG and heart transplantation could take place. Three other affected children with mild dilated cardiomyopathy at the time of the diagnosis deteriorated rapidly, two of them within days after an acute infection. They all went through successful heart transplantation; one died unexpectedly and 2 others are currently (after 1–5 years) clinically stable. The other 4 children diagnosed with mild dilated cardiomyopathy are doing well on supportive heart failure therapy. In most cases, the cardiac findings dominated the clinical picture, without central nervous system or multisystem involvement, which is unique in CDG syndrome. We suggest to test for DK1-CDG in patients with dilated cardiomyopathy. Patients with discrete cardiomyopathy may remain stable on supportive treatment while others deteriorate rapidly. Our paper is the first comprehensive study on the phenotype of DK1-CDG and the first successful organ transplantation in CDG syndrome.     

Children may survive severe myocarditis with prolonged use of biventricular assist devices

The outcome of acute myocarditis with cardiogenic shock is poor. In some children in whom aggressive medical treatment fails, artificial replacement of heart function may offer lifesaving support until the myocardium has recovered. Four previously healthy children (three boys aged 4, 6, and 1 years; one girl aged 5) developed acute myocarditis with ventricular failure and multiorgan dysfunction caused by low cardiac output. Biventricular assist devices (BVAD) were implanted for prolonged support. In three children cardiac function improved and after up to 21 days mechanical support could be withdrawn. They had full recovery of heart function. In the fourth patient there was no myocardial recovery after a period of 20 days. He underwent orthotopic heart transplantation with an uneventful postoperative course. Prolonged circulatory support with BVAD is an effective method for bridging until cardiac recovery or transplantation in children.     

Brief Report: Antisynthetase Syndrome–Associated Myocarditis

BackgroundThe antisynthetase (AS) syndrome is characterized by autoimmune myopathy, interstitial lung disease, cutaneous involvement, arthritis, fever, and antibody specificity. We describe 2 patients with AS syndrome who also developed myocarditis, depressed biventricular function, and congestive heart failure.Methods and ResultsBoth patients were diagnosed with AS syndrome based on clinical manifestations, detection of serum AS antibodies, and myositis confirmation with the use of skeletal muscle magnetic resonance imaging and skeletal muscle biopsy. In addition, myocarditis resulting in heart failure was confirmed with the use of cardiac magnetic resonance imaging and from endomyocardial biopsy findings. After treatment for presumed AS syndrome–associated myocarditis, one patient recovered and the other patient died.ConclusionsAS syndrome is a rare entity with morbidity and mortality typically attributed to myositis and lung involvement. This is the first report of AS syndrome–associated myocarditis leading to congestive heart failure in 2 patients. Given the potentially fatal consequences, myocarditis should be considered in patients with AS syndrome presenting with heart failure.     

Biventricular Assist Device Terminates Polymorphic Ventricular Tachycardia in Giant Cell Myocarditis

We present the case of a 55-year-old woman with giant cell myocarditis who experienced a rapid deterioration in her condition. As her heart failure progressed, she developed more ventricular ectopic beats, which culminated in a polymorphic ventricular tachycardia that did not improve despite immunosuppressive and antiarrhythmic therapy. Emergent biventricular assist device placement, however, did eliminate her arrhythmia.Key words: Giant cells/pathology, heart-assist devices, myocarditis/complications/diagnosis/physiopathology, tachycardia, ventricular/etiology/physiopathologyGiant cell myocarditis (GCM) is a rare form of cardiomyopathy with a high mortality rate. In the largest GCM series published to date (63 patients),¹ the rate of death or cardiac transplantation was 89%, with a median survival of 5.5 months from the onset of symptoms to the time of death or transplantation. Patients with GCM manifest a diffuse, infiltrative disease process and often have conduction system abnormalities such as heart block, bundle branch block, and ventricular tachycardia (VT). We present the case of a patient, diagnosed with GCM, whose ventricular arrhythmias were refractory to medical management but resolved after mechanical circulatory support.     

The value of follow-up after acute Q fever infection

This is a case report of a 53-year-old woman involved in an outbreak of Q fever, in whom Q fever endocarditis was diagnosed 18 months after acute Q fever infection. At the time of diagnosis, she was completely asymptomatic and without screening for chronic Q fever, this severe potentially life-threatening infection would probably not have been recognised until significant valvular destruction had taken place. Early diagnosis enabled prompt, potentially curative medical treatment to start without the need for valvular heart surgery. The authors advocate that serological monitoring should be carried out every 4 months for a period of 2 years after acute Q fever and patients with high phase 1 IgG titres (>800) be investigated further and/or followed more closely depending on the clinical scenario. The case report also discusses the use of complement fixation testing in the diagnosis of Q fever endocarditis. The authors recommend that in cases of culture negative endocarditis, a single negative complement fixation test is not sufficient to exclude the diagnosis of Q fever endocarditis. Micro-immunofluorescence or repeat complement fixation testing is recommended when Q fever endocarditis is suspected clinically.     

A loud third heart sound and asymptomatic myocarditis during Mycoplasma pneumoniae infection

A 20-year-old man had a fever and cough due to Mycoplasma pneumoniae pneumonia. He had no heart symptoms, but auscultation revealed an exceptionally loud third heart sound, suggesting cardiac involvement. Marked myocardial enzyme release, serial electrocardiographic ST-T changes, and transient increase in interventricular thickness and inferior wall hypokinesis at echocardiography supported the diagnosis of acute infectious myocarditis. Recovery was quick. This case shows that acute myocarditis with significant myocardial injury may pass without any subjective heart symptoms.     

Cardiac findings in childhood staphylococcal sepsis

The clinical and laboratory findings of eight (20%) cases of cardiac involvement of 39 patients with sepsis caused by S. aureus (Staphylococcus aureus) were reviewed retrospectively. Our purpose was to emphasize the importance of the cardiac findings in patients with sepsis caused by S. aureus in childhood. The ages of the patients ranged from 6 to 14 years. All patients had pericardial effusion which was confirmed by echocardiographic (ECHO) examination in all cases except the one in whom ECHO examination could not be performed because he died 2.5 days after admission to the hospital. This patient also had myocarditis and heart failure. Aside from these, mitral insufficiency was diagnosed in the other patient; it was accepted as a sequela of rheumatic fever acquired previously. Open pericardial drainage was conducted successfully in the case who had a progression to cardiac tamponade. In the other patients pericardial effusion completely resolved with supportive and antibiotic therapy one to two weeks. Two of eight patients died from sepsis and septic shock; the mortality rate was 25%. Our findings show that cardiac involvement was fairly high (20%) in S. aureus sepsis in childhood. Therefore, it is suggested that children with S. aureus sepsis should be carefully monitored for cardiac involvement.     

Support with the BVS 5000 assist device during treatment of acute giant-cell myocarditis

Giant-cell myocarditis is a rare and aggressive form of myocarditis with a high mortality rate. Our purpose is to summarize 3 cases of acute giant-cell myocarditis that illustrate possible outcomes with mechanical support. We reviewed the cases of 3 patients, aged 39 to 59 years, who had giant-cell myocarditis (confirmed by myocardial biopsy). The indication for ventricular assist was circulatory failure despite maximal medical treatment with 2 or more inotropic agents and intraaortic balloon pump support. Immunosuppression and a biventricular mechanical assist (BVS 5000) were used to treat all these patients. The mean duration of mechanical support was 15.7 days (range, 10 to 19 days). One patient had recovery of myocardial function and was weaned from mechanical support. This case is, to our knowledge, the first reported of ventricular support leading to cardiac recovery after diagnosis of giant-cell myocarditis. The 2nd patient was not a candidate for further surgery and died of multisystem organ failure. The 3rd patient underwent orthotopic heart transplantation after 18 days of support and was discharged. We conclude that patients with giant-cell myocarditis tend to have biventricular involvement and can recover myocardial function on mechanical support and immunosuppression. If recovery is not observed, transplantation is warranted. By avoiding left ventricular cannulation, the BVS 5000 is well suited for bridging to recovery, transplantation, or long-term support.     

Clinical outcomes of acute myocarditis in childhood

OBJECTIVE: To describe clinical outcomes of a paediatric population with histologically confirmed lymphocytic myocarditis. DESIGN: A retrospective review between November 1984 and February 1998. SETTING: A major paediatric tertiary care hospital. PATIENTS: 36 patients with histologically confirmed lymphocytic myocarditis. MAIN OUTCOME MEASURES: Survival, cardiac transplantation, recovery of ventricular function, and persistence of dysrhythmias. RESULTS: Freedom from death or cardiac transplantation was 86% at one month and 79% after two years. Five deaths occurred within 72 hours of admission, and one late death at 1.9 years. Extracorporeal membrane oxygenation support was used in four patients, and three patients underwent heart replacement. 34 patients were treated with intravenous corticosteroids. In the survivor/non-cardiac transplantation group (n = 29), the median follow up was 19 months (range 1.2-131.6 months), and the median period for recovery of a left ventricular ejection fraction to > 55% was 2.8 months (range 0-28 months). The mean (SD) final left ventricular ejection and shortening fractions were 66 (9)% and 34 (8)%, respectively. Two patients had residual ventricular dysfunction. No patient required antiarrhythmic treatment. All survivors reported no cardiac symptoms or restrictions in physical activity. CONCLUSIONS: Our experience documents good outcomes in paediatric patients presenting with acute heart failure secondary to acute lymphocytic myocarditis treated with immunosuppression. Excellent survival and recovery of ventricular function, with the absence of significant arrhythmias, continued cardiac medications, or restrictions in physical activity were the normal outcomes.     

Role of right ventricular endomyocardial biopsy in infants and children with suspected or possible myocarditis.

OBJECTIVES--To assess the diagnostic yield, sampling errors, risks, and therapeutic implications of right ventricular endomyocardial biopsy in children with suspected or possible myocarditis. DESIGN--Retrospective study. SETTING--Tertiary referral centre for paediatric cardiology, cardiac surgery, heart transplantation, and mechanical circulatory support. PATIENTS AND METHODS--Review of clinical and histological findings among 63 consecutive children with possible myocarditis undergoing right ventricular endomyocardial biopsy. Review of cardiac histology at subsequent necropsy or after explantation at time of transplantation. RESULTS--From January 1980 to December 1992, 76 biopsies were performed in 63 children (2 weeks to 18 years of age). In 41 cases, the biopsy was performed for evaluation of dilated cardiomyopathy. The median interval from onset of symptoms was one month. Eight children (20%; all with a history of less than six weeks duration) had biopsy proved myocarditis. Five of the eight children made a full recovery, including four who presented in cardiogenic shock. By contrast, only three of 33 children without evidence of myocarditis showed recovery of ventricular function. The whole heart was available for histological examination in 23 patients. Myocarditis was confirmed in one patient, and no evidence of myocarditis was found in the remaining 22 (all with negative biopsies). One procedure related death occurred in a 2 week old infant with dilated cardiomyopathy. In 22 cases, biopsy was performed for the evaluation of arrhythmia. Only one biopsy showed myocarditis. CONCLUSIONS--The diagnostic yield of a biopsy is low in children with arrhythmias. In children presenting with profound ventricular dysfunction, a diagnosis of acute myocarditis may avoid premature consideration of transplantation as this group has an important potential for full recovery. In less critically ill patients and in those with a longer duration of symptoms the justification for biopsy is not as clear and the procedure is not without risk.     

Determinants for clinical diagnosis of hypertrophic cardiomyopathy

Although hypertrophic cardiomyopathy (HC) occurs in 1 of 500 adults, most cardiology practices treat relatively few patients with HC, suggesting that many affected patients evade clinical recognition. Determining the clinical circumstances under which HC is identified will provide clues to its under-recognition. Clinical triggers leading to diagnostic echocardiograms were analyzed in 711 consecutive patients with HC. In most (384 [54%]), HC was initially suspected only after the onset of cardiac symptoms or acute cardiac events. In a substantial minority (327 [46%]), HC was recognized while patients were asymptomatic, including 225 (32%) by routine medical evaluations, in 27 of whom (4%) HC was recognized during preparticipation examinations for competitive sports or other activities. Women, older patients (age > or =50 years), and those with outflow obstruction at rest (gradient > or =30 mm Hg) were more likely suspected to have HC by virtue of cardiac symptoms or events (p <0.0001). Conversely, patients with extreme hypertrophy (wall thickness > or =30 mm) and those at high risk for sudden death were more often asymptomatic and identified by routine or family screenings (p <0.0001 and p = 0.004, respectively). Patients who subsequently died of heart failure or experienced embolic stroke were more often identified by virtue of symptoms or acute events (p = 0.03). In conclusion, although most patients with HC were recognized clinically only after overt disease manifestations, a substantial minority were diagnosed by routine examinations while asymptomatic, including an important subset of patients with HC recognized solely because of findings on sports preparticipation screening. These data underscore the need for heightened awareness and clinical suspicion of HC to increase the number of diagnosed patients, including many who may be at high risk for sudden death.     

Acute Q Fever: 35 cases in Castilla-La Mancha

We report 35 sporadic cases of acute Q fever diagnosed in the area of Castilla-La Mancha (Spain). Diagnosis was based on a fourfold or greater rise in specific antibody titer. The mean age of the patients was 33 years and the male/female ratio was 2.5. Seventeen patients had hepatitis, 9 had pneumonia, 8 had isolated fever and 1 had myocarditis. An underlying disease was more frequent among patients with pneumonia.     

Pharmaceutical management of decompensated heart failure syndrome in children: Current state of the art and a new approach

Prompt initiation of appropriate and intensive treatment in children with decompensated heart failure is crucial to avoid irreversible end-organ dysfunction. Initial management of these children includes transfer to the pediatric cardiac intensive care unit, basic hemodynamic monitoring, and establishment of intravenous access. Inotropic support should be instituted peripherally before obtaining central venous and arterial access. The team should be prepared for emergent intubation and initiation of mechanical circulatory support. Two experienced physicians should work together to obtain vascular access and manage sedation, airway control, and cardiovascular support. Acute heart failure syndrome in children may be related to cardiomyopathy, myocarditis, congenital heart disease, and acute rejection post heart transplantation. Each of these causes requires a different approach. Fulminant myocarditis may lead to severe morbidity and requires intensive support, although its outcome is considered to be good. Acute heart failure related to newly diagnosed dilated cardiomyopathy may represent end-stage heart failure; therefore, long-term mechanical circulatory support and heart transplantation may be considered to avoid other end-organ dysfunction. Hypertrophic cardiomyopathy may lead to acute decompensation due to 1) left ventricular outflow obstruction, 2) restrictive physiology leading to pulmonary hypertension, or 3) myocardial is chemia associated with coronary artery bridging. Decompensated heart failure associated with congenital heart disease usually represents end-stage heart failure and requires thorough evaluation for heart transplantation. Children with single-ventricle physiology who develop decompensated heart failure after a Fontan procedure are not candidates for mechanical circulatory support and therefore may not survive to heart transplantation. Acute heart failure due to posttransplantation acute rejection requires aggressive antirejection treatment, which places these patients at significant risk for overwhelming opportunistic infections. In our opinion, mechanical circulatory support should be initiated early in children who present with end-stage heart failure associated with hemodynamic instability to avoid end-organ damage.