Atrial septal defect in adults is associated with airway hyperresponsiveness

Objective: The association between secundum atrial septal defects (ASD) and asthma-like dyspnea with consequent long-term pulmonary inhalant use, is poorly understood in adult ASD patients. Airway hyperresponsiveness is suggested to be the underlying mechanism of cardiac asthma from mitral valve disease and ischemic cardiomyopathy. We hypothesized that airway hyperresponsiveness may also be found in adult ASD patients. Our aim was to study airway responsiveness in adult ASD patients before percutaneous closure and at short-and long-term postprocedural follow-up. Methods: This prospective study included 31 ASD patients (65% female, mean age 49 ± 15y) who underwent spirometry and bronchoprovocation testing pre-and six-month postprocedurally, with additional bronchoprovocation at 2-year follow-up. Airway hyperresponsiveness was defined as ≥20% fall of forced expiratory volume in 1-second (FEV1) following <8.0 mg/mL of inhaled methacholine. Results: Airway hyperresponsiveness was found in 19/30 patients (63%[95%CI 45%-81%]; post hoc statistical power = 89%). Asthma-like symptoms wheezing, chest tightness, and cough were more frequently reported in airway hyperresponsive patients. Airway responsiveness was not influenced by successful percutaneous ASD closure, corresponding to persistence of asthma-like symptoms postclosure. Regardless of airway responsiveness, postprocedural right-sided reverse remodeling significantly improved dyspnea and pulmonary function. Conclusions: This study is the first to report a high prevalence of airway hyperresponsiveness in a cohort of unrepaired adult ASD patients, and confirms the association between asthma-like symptoms and ASD in adults. Attention to symptoms and pulmonary function should be given during clinical follow-up of adult ASD patients, both before and long after repair

Successful IVIG Treatment of Human Parechovirus-Associated Dilated Cardiomyopathy in an Infant

Human parechoviruses (HPeVs) are closely related to human enteroviruses and exhibit many similarities in disease spectrum and symptoms. HPeV1 is most commonly associated with mild disease, but rare associations with severe disease such as myocarditis have been reported. Currently, no treatment is available for severe HPeV infections. In this case report we describe an infant with a severe, dilated cardiomyopathy in whom HPeV1 was revealed to be the only identifiable cause. The infant was treated with intravenous immunoglobulins (IVIGs) and recovered completely. In vivo blood samples revealed a high HPeV1 antibody titer after treatment with IVIGs. In vitro IVIGs contained high titers of neutralizing antibodies against HPeV1. Our hypothesis is that patients with myocarditis caused by viruses with a high prevalence in the population and hence high antibody titers in IVIGs are likely to benefit from treatment with IVIGs. More research combining virological and clinical data is needed to see whether this hypothesis is tru

Left ventricular ischemia after arterial switch procedure: Role of myocardial perfusion scintigraphy and cardiac CT

Transposition of the great arteries is a congenital heart defect defined by an abnormal connection between the aorta, pulmonary artery, and the ventricles, resulting in parallel systemic and pulmonary circulations. Long-term follow-up data of patients who underwent correction via an arterial switch operation have recently shown that as a result of re-implantation of the coronary arteries in the neo-aorta, coronary stenosis and occlusion are relatively common complications. In this report, we discuss two cases illustrating the added value of myocardial perfusion imaging (MPI) and cardiac CT for the assessment of these patients. Based on the available literature we conclude that MPI and cardiac CT are excellent non-invasive methods to evaluate coronary anatomy and myocardial function also in this specific group of patients

Intravenous immunoglobulins in children with new onset dilated cardiomyopathy

Background: Dilated cardiomyopathy is a rare but serious disorder in children. No effective diagnostic or treatment tools are readily available. This study aimed to evaluate the efficacy of intravenous immunoglobulins in children with new onset dilated cardiomyopathy. Methods and results: In this retrospective cohort study, 94 children with new onset dilated cardiomyopathy were followed during a median period of 33 months. All patients with secondary dilated cardiomyopathy - for example, genetic, auto-immune or structural defects - had been excluded. Viral tests were performed in all patients and 18 (19%) children met the criteria for the diagnosis "probable or definite viral myocarditis". Intravenous immunoglobulins were administered to 21 (22%) patients. Overall transplant-free survival was 75% in 5 years and did not differ between treatment groups. The treatment was associated with a higher recovery rate within 5 years, compared with non-treated children (70 versus 43%, log rank = 0.045). After correction for possible confounders the hazard ratio for recovery with intravenous immunoglobulins was not significant (hazard ratio: 2.1; 95% CI: 1.0-4.6; p = 0.056). Administration of intravenous immunoglobulins resulted in a greater improvement in the shortening fraction of the left ventricle. Conclusion: In our population of children with new onset dilated cardiomyopathy, of either viral or idiopathic origin, intravenous immunoglobulins were administered to a minority of the patients and did not influence transplant-free survival, but were associated with better improvement of systolic left ventricular function and with better recovery. Our results support the concept that children with new onset dilated cardiomyopathy might benefit from intravenous immunoglobulin

Een meisje met dyspneu en een vergrote lever

BACKGROUND: During winter, the influenza and Respiratory Syncytial Virus season, children are often seen in the emergency department with fever and respiratory symptoms. CASE DESCRIPTION: We describe the case of a 2-year-old girl with fever, dyspnea and signs of heart failure during physical examination. Echocardiogram showed a large amount of pericardial effusion with signs of cardiac tamponade. Pericardiocentesis showed 200 ml purulent liquid. Surgical drainage was performed. However pericardial effusion recurred. Because constrictive pericarditis was feared, a pericardiectomy was performed after which the patient recovered. Cultures showed Haemophilus influenzae type b and the patient was treated with amoxicillin for 4 weeks in total. CONCLUSION: Fever and dyspnea may also be caused by a problem outside the respiratory system. With physical examination a cardiac cause, such as pericarditis, can be detected. An ECG is easily obtained and can help in the differential diagnosis. An echocardiogram can confirm the diagnosis

Een meisje met dyspneu en een vergrote lever

BACKGROUND: During winter, the influenza and Respiratory Syncytial Virus season, children are often seen in the emergency department with fever and respiratory symptoms. CASE DESCRIPTION: We describe the case of a 2-year-old girl with fever, dyspnea and signs of heart failure during physical examination. Echocardiogram showed a large amount of pericardial effusion with signs of cardiac tamponade. Pericardiocentesis showed 200 ml purulent liquid. Surgical drainage was performed. However pericardial effusion recurred. Because constrictive pericarditis was feared, a pericardiectomy was performed after which the patient recovered. Cultures showed Haemophilus influenzae type b and the patient was treated with amoxicillin for 4 weeks in total. CONCLUSION: Fever and dyspnea may also be caused by a problem outside the respiratory system. With physical examination a cardiac cause, such as pericarditis, can be detected. An ECG is easily obtained and can help in the differential diagnosis. An echocardiogram can confirm the diagnosis