Bulging pericardium may also be present. Most common associated congenital defects are endocardial cushion defect and mitral regurgitation. VSD, TOF, PDA, CoA, AI, and ASD may be present. Also, pulmonary hypertension and CHF are present. Inadequate alveolarization is found in terminal lung units distal to respiratory bronchioles, total decrease in cross sectional area of vascular bed, pulmonary vascular disease is prevalent.
Skin tends to be dry and relatively pale.
Mouth sometimes turns down, more common in females. Moro reflex - defensive reflex where the infant draws its arms across its chest in response to stimuli. Surgical Intervention Limited to correction of congenital defects, survival appears to be no more different from other patients with similar defects.