Description of cases
Case 1: 4-year-old male, with a history of respiratory tract
infection and heart murmur, diagnosed a year before with patent ductus
arteriosus and absence of RPA (Figure 1A ). Physical examination
revealed a continuous murmur in the pulmonary site. The echocardiogram
showed a wide ductus arteriosus, a dilated MPA continuing with left
pulmonary artery (LPA) and absence of connection with the RPA.
Cardiac catheterization showed pulmonary hypertension and double ductus
arteriosus with a discontinuous pulmonary artery, where the MPA
continued with the LPA while the RPA was hypoplastic with an anomalous
origin in a right filiform ductus arteriosus emerging from the
brachiocephalic trunk. The left ductus arteriosus was closed with a
ductal occluder device and a percutaneous transluminal angioplasty with
placement of a right intraductal stent was performed (Figure
1B-C ).
At one year of follow-up, normal pulmonary pressure was verified with
adequate development of RPA (Figure 2A-2B ), deciding upon a
surgical correction with cardiopulmonary bypass without aortic cross
clamping, with surgical closure of the right ductus arteriosus and
connection of RPA to MPA with a Gore-Tex® 12 mm graft (Figure
2C ).
Case 2: 10-year-old female, with a history of fatigue,
diaphoresis and continuous murmur in the pulmonary site.
Echocardiography and computed tomography showed persistence of the
ductus arteriosus and absence of RPA. Cardiac catheterization showed a
double ductal system, hypoplastic RPA originating from the right ductus
arteriosus emerging from the brachiocephalic trunk, a wide left ductus
arteriosus and MPA continuing with LPA (Figure 3A );
additionally, an aortopulmonary collateral to the right lung and
pulmonary hypertension was diagnosed. Percutaneous transluminal
angioplasty was performed with placement of a right intraductal stent,
transcatheter embolization of the aortopulmonary collateral with
vascular plug and closure of the left ductus arteriosus with a ductal
occluder device. One year later, she presented with proximal stenosis of
the right ductus arteriosus for which a percutaneous transluminal
balloon angioplasty was performed.
At 7 years of follow-up, she presented with normal pulmonary artery
pressure and adequate development of RPA (Figure 3B ),
performing a surgical repair with cardiopulmonary bypass without aortic
cross clamping, connection of RPA to MPA with a Gore-Tex® 14 mm graft,
in addition to the surgical closure of the right ductus arteriosus. Both
patients presented a favorable postoperative outcome.