We describe an infant with congenital mydriasis, patent ductus arteriosus (PDA), pulmonary hypertension, and cystic lung disease. She had all the major components of multisystemic smooth muscle dysfunction syndrome. Due to progressive respiratory deterioration, she required surgical PDA interruption, extracorporeal life support, and subsequent prolonged respiratory support. Genetic testing revealed ACTA2 R179H mutation and cystic lung disease on biopsy.

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Values for sensitivity and specificity indicate that telephone transmission is a useful diagnostic tool for assessing pacemaker function at a distance. Negative predictive value is 99.9%, indicating that normal telephone transmissions are very reassuring of normal pacemaker function. Telephone transmission is equally successful in all age groups, genders, distances from a tertiary referral center, underlying diagnoses, pacing modes, and pacemaker models. Compliance with telephone transmission follow-up was higher in our population than in previous studies.

After repair of an atrioventricular septal defect with common atrioventricular junction in a 2-month-old girl, rapid atrial tachycardia, in combination with junctional ectopic tachycardia, led to severe postoperative cardiovascular compromise. Intercurrent runs of ectopic atrial tachycardia made atrial pacing impossible, despite high doses of intravenous amiodarone. Following the addition of flecainide to the infusion, we were able to control the rhythm, and when combined with atrial pacing, this led to an immediate haemodynamic improvement. Treatment of refractory supraventricular tachycardias with amiodarone combined with flecainide can be very effective in the setting of postoperative cardiac intensive care.

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