IFX therapy was observed to be effective for the early improvement of CAAs in patients with intravenous immunoglobulin-resistant KD.

No abstract

Isolated unilateral pulmonary venous atresia (PVA) is an extremely rare anomaly that typically presents with hemoptysis and pneumonia; however, the most appropriate management strategy remains controversial due to the high variability in clinical course between cases. Herein, we present three cases and discuss the diagnostic and therapeutic considerations. Case 1: A 4-year-old girl presented with a history of recurrent hemoptysis and pneumonia since 18 months of age. Lung perfusion scintigraphy demonstrated decrease in the right pulmonary blood flow. Pulmonary arterial wedge angiography (PAWA) identified a right PVA. The patient underwent pulmonary vein reconstruction using a sutureless technique. Case 2: A 4-year-old girl was referred due to hemoptysis. Lung perfusion scintigraphy demonstrated a decrease in left pulmonary blood flow, and the diagnosis was confirmed by PAWA. We abandoned the repair in this case due the presence of a long gap between the left pulmonary vein and left atrium. Case 3: A 36-year-old female presented with a history of recurrent hemoptysis and pneumonia from infancy. PVA was suspected according to the results of lung perfusion scintigraphy, and the diagnosis was confirmed by PAWA. We abandoned the repair in this case as the development of pulmonary arterial obstructive disease was expected. If lung perfusion scintigraphy demonstrates a remarkable reduction in unilateral pulmonary blood flow, PVA should be suspected and PAWA should be performed to confirm the diagnosis. Pulmonary vein reconstruction represents a treatment option in cases where the diagnosis is made in early childhood and anatomical repair is feasible. For right PVA, the sutureless technique is considered to be effective in preventing postoperative pulmonary venous stenosis.

We present a neonatal girl who developed complete atrioventricular block after postnatal cardioversion for fetal ventricular tachyarrhythmia. A pregnant women was referred at 39 gestational weeks due to fetal tachyarrhythmia of 180 bpm and without atrioventricular (AV) dissociation. Subsequently, a baby girl was delivered weighing 3,130 g. Electrocardiography revealed wide QRS tachycardia with left axis deviation, and the esophageal leads revealed a short RP′ duration without AV dissociation. However, AV dissociation was overt after injecting adenosine triphosphate, which suggested ventricular tachycardia. Cardioversion stopped the ventricular tachycardia but led to atrioventricular block with fascicular block (P rate 138 bpm, QRS rate 75 bpm), and the resulting hypotension (32/15 mmHg) was improved by dopamine administration. Echocardiography revealed a left ventricular end-diastolic diameter of 16.8 mm and an ejection fraction of 30. Serial electrocardiography revealed a second-degree atrioventricular block at 15 hours after birth that reverted to sinus rhythm at day 2 after birth. She was discharged on day 48 after birth. Holter electrocardiographic monitoring did not shown recurrence of either the ventricular tachycardia or the atrioventricular block. The present case was unique because advanced atrioventricular block due to overdrive suppression in the His Purkinje conduction system followed ventricular tachycardia with ventriculoarterial conduction.