Table II.

Clinical characteristics of infants with atypical presentation of ACDMPV17, 19

Patients	EGA (wks)	Neonatal symptoms	Age at presentation	Cardiac anomalies	Additional anomalies	CT imaging	Cardiac catheterization	Pulmonary hypertension medications	ECMO	Support at time of lung transplantation	Age at transplantation (mo)	Outcome
1	Term	Transient tachypnea at birth, discharged home on room air	3 months (109 days)	None	Inguinal hernia	Not available	Not performed	Prostacyclin, iNO	Before transplant	Mechanical ventilation	4	Second transplant at 4 years of age; died at 5 years of age
2	Term	Respiratory distress, pulmonary hypertension	Birth	Patent foramen ovale	Posterior urethral valves	Ground glass opacities, enlarged pulmonary vessels	Suprasystemic RV pressure, responsive to iNO	Nifedipine, iNO	No	NO and O2 via nasal cannula	20	Second transplant at 4 years of age; alive at 16 years of age
3	36	None	2 months (71 days)	Patent foramen ovale	None	Diffuse ILD, associated cystic areas	Not performed	Milrinone, methylprednisolone, iNO	No	Mechanical ventilation	5	Alive at 14 years of age
4	34	Poor feeding, abdominal distension at 3 days	2 months (67 days)	Secundum atrial septal defect	None	Ground glass opacities, interstitial septal thickening	Suprasystemic RV pressure, responsive to iNO	Sildenafil, milrinone, iNO	No	Mechanical ventilation	5	Died at 9 years of age
5	34	Apnea at 3 weeks	3 months (92 days)	Patent foramen ovale	None	Ground glass opacities	Not performed	Sildenafil Prednisolone, iNO	At transplantat	ECMO	9	Alive at 5 years of age
6	Term	Transient tachypnea, discharged home on O2 for 21 days	7 months (212 days)	Patent ductus arteriosus	None	Ground glass opacities with septal thickening	Near systemic RV pressure, responsive to iNO	Sildenafil, methylprednisolone, iNO	Before transplantat	Positive pressure ventilation via RAM cannula	15	Died at 18 months of age

ECMO, extracorporeal membrane oxygenation; EGA, Estimated gestational age; iNO, inhaled nitric oxide; RAM, ; RV, right ventricular.