ABSTRACT
Two previously healthy infants, a boy and a girl aged 3 and 4 months, respectively, were admitted for fever of unknown origin with laboratory results indicating an inflammation. The boy presented with vomiting, pyuria, anaemia, and thrombocytosis. The girl presented with irritability, erythema and diarrhoea. All viral and bacterial cultures remained negative and supplementary radiology was unable to detect a focus of infection. The fever had persisted for at least 12 days in both cases before the diagnosis 'atypical Kawasaki disease' was considered. Cardiac echograms showed dilatation of the coronary arteries in both patients and confirmed the diagnosis. Immediate therapy with intravenous immunoglobulins and acetylsalicylic acid was given, whereupon the fever subsided within 24 hours; the further clinical course was uneventful. These cases illustrate the fact that atypical Kawasaki disease is often a late consideration, especially when the symptoms of the classical form are absent. This condition should be considered in every infant presenting with long-lasting unexplained fever.
Subject(s)
Fever of Unknown Origin/etiology , Mucocutaneous Lymph Node Syndrome/diagnosis , Anti-Inflammatory Agents, Non-Steroidal/therapeutic use , Aspirin/therapeutic use , Coronary Vessels/diagnostic imaging , Coronary Vessels/pathology , Diagnosis, Differential , Echocardiography , Female , Humans , Immunoglobulins, Intravenous/therapeutic use , Infant , Male , Mucocutaneous Lymph Node Syndrome/complications , Mucocutaneous Lymph Node Syndrome/diagnostic imaging , Mucocutaneous Lymph Node Syndrome/therapy , Treatment OutcomeABSTRACT
BACKGROUND: Correction of tetralogy of Fallot (ToF) often leads to pulmonary regurgitation, sometimes warranting pulmonary valve replacement (PVR), for which the indications and timing to achieve optimal results are not yet clear. This retrospective study describes follow-up and reinterventions in our ToF population. METHODS: Review of all consecutive patients operated for ToF between 1977 and 2000. Included are date and type of repair, Doppler echocardiography (2D-echo), ECGs, re-operations and physical condition. RESULTS: Total repair was performed in 270 patients, mean age 1.9±2.5 years, 82 were excluded because of follow-up abroad. Right ventriculotomy was used in 92%, transatrial VSD closure in 8%, while 69% received a transannular outflow patch. Pulmonary atresia required a pulmonary graft in 13 (8%) patients. Overall 20-year survival was 88%. Last follow-up: ECG showed RBBB in 67% (QRS complex 129±29.3 msec). RVOT aneurysms were detected in 16%. 2D-echo demonstrated mild pulmonary insufficiency (PI) in 40%, severe in 31%, dilated RV in 76%, both increasing with post-repair age. In 39%, RV dimensions were equal or even exceeded LV dimensions, 45% showed tricuspid insufficiency and the RA was enlarged in 14%. Reintervention was necessary in 39/185 patients, this included angioplasty for residual stenosis and PVR (22/19 homografts, six patients in PA group) at a mean age of 11.2 years after correction. In seven patients, the RV returned to normal dimensions and symptoms disappeared, but in three severe dysfunction developed. Eleven others still have RV dilatation and/or PI. In total, 75% were free of reintervention in the first ten years. The right atrial approach diminishes severe RV dilatation and prolonged QRS duration (p=0.001 and 0.007). Early correction reduces the risk of re-operation (p=0.011). CONCLUSIONS: Severe RV dilatation (39%) and PI (31%) secondary to outflow tract repair in ToF are frequently occurring sequels developing slowly over time. Timing of PVR remains controversial, still best guided by the clinical condition.
