Familial Pulmonary Capillary Hemangiomatosis Early in Life

Table 1

Clinical characteristics of the 3 patients with PCH.

	Patient 1	Patient 2	Patient 3
Gestational age (wks.)	42	38	15
Malformations	—	Umbilical hernia; diastasis m. recti; gothic palate	—
Onset of symptoms	1st day of life	3 months	—
Pulmonary symptoms	Supplemental oxygen need since birth	Recurrent obstructive bronchitis in infancy	—
Patent ductus arteriosus	Surgical closure in neonatal period	Surgical closure at the age of 7 months	Prenatal status
Mechanical ventilation	Ventilated for almost entire life span	Only used for elective surgery	—
Pulmonary hypertension	Confirmed during heart catheterization	Suspected by echocardiography and electrocardiography	—
Outcome	Death at the age of 5 months due to respiratory failure	Poor growth during first years; recurrent; obstructive bronchitis; neuro-developmental delay	Abortion