His bundle pacing in a young child guided by electroanatomical mapping.

Pediatric patients with complete congenital atrio-ventricular (AV) block are generally exposed to life-long dyssynchronous right ventricular (RV) pacing. His bundle pacing (HBP) is an alternative method of pacing that better restores physiological ventricular activation which could prevent pacing-induced cardiomyopathy. We present a case of a 5-year-old child with complete AV block who underwent successful permanent HBP implantation. Three-dimensional electro-anatomical mapping system was used to facilitate the procedure and reduce the fluoroscopy time. There were no acute procedure-related complications, and electrical parameters were stable at short-term follow-up.

A Scimitar Syndrome Variant Associated with Critical Aortic Coarctation in a Newborn.

BACKGROUND Scimitar syndrome (SCS) is a rare congenital cardiopulmonary malformation, characterized by anomalous pulmonary venous drainage from the right lung associated with aortopulmonary collateral arteries and pulmonary hypoplasia. The variant described in the case presented here, with total anomalous right pulmonary venous drainage into the superior and inferior vena cava, can be expected in 2% of patients with scimitar syndrome. To the best of our knowledge, the association between the variant of SCS and coarctation of aorta described in our patient has never been reported before in the literature. CASE REPORT A female newborn with a gestational age of 35 weeks presented with a rare combination of scimitar syndrome and aortic coarctation. The patient had a variant of SCS that included anomalous drainage of the right upper and lower pulmonary vein into the superior and inferior vena cava, respectively; relative right lung hypoplasia; and right lower lobe sequestration supplied by aortopulmonary collateral arteries that originated from the truncus coeliacus. The diagnosis was confirmed with computed tomography angiography after resection of the aortic coarctation with extended end-to-end anastomosis. Subsequently, interventional closure of the collateral artery supplying the right lower lobe was performed with an AMPLATZER™ Vascular Plug 4. The patient's clinical course was complicated by suspicious acute endocarditis and chylous pleural effusion. After a prolonged hospitalization, she was discharged in clinically stable condition. CONCLUSIONS The diagnosis of SCS should be considered when pulmonary hypertension persists after coarctation repair in a child with dextroposition of the heart and right lung hypoplasia. Successful treatment of this rare combination of conditions calls for teamwork by highly experienced specialists.