We presented nine non-operated and six operated patients of our center. The APAs were LPAs in all 15 cases. Seven patients were male, and eight were female.
We decided to find and open the hidden pulmonary artery of all the nine patients, which was successful in three cases. We could not open the pulmonary artery of four cases, and two of them had a good-sized concealed pulmonary artery via pulmonary vein angiography and did not require pulmonary artery rehabilitation (Table 1).
Table 1 Demographic data of the nine non-operated patients with a hidden pulmonary
Patients
(Number)
|
Patients
(Group)
|
Age
(Month)
|
Weight
(kg)
|
Diagnosis
|
Aortic arch
|
Hidden pulmonary artery
|
1
|
Group 1
|
1
|
3.5
|
TOF
|
Right
|
Left
|
2
|
Group 1
|
24
|
10.5
|
TOF
|
Right
|
Left
|
3
|
Group 1
|
30
|
12
|
TOF, APV
|
Right
|
Left
|
4
|
Group 2
|
10
|
8.5
|
TOF
|
Left
|
Left
|
5
|
Group 2
|
15
|
9.5
|
TOF
|
Left
|
Left
|
6
|
Group 3
|
12
|
7.5
|
TOF, APV
|
Left
|
Left
|
7
|
Group 3
|
38
|
13.5
|
TOF
|
Right
|
Left
|
8
|
Group 3
|
60
|
15.5
|
TOF
|
Left
|
Left
|
9
|
Group 3
|
63
|
16
|
TOF
|
Left
|
Left
|
APV, absent pulmonary valve; F, female; M, male; Mo, month; No, number; TOF, tetralogy of Fallot
Totally eleven cases had left aortic arch, and four had the right aortic arch.
Nine DAs were not in their usual site and came from underneath the aortic arch or the beginning of the brachiocephalic artery, but the DA in six cases was in its proper place.
Two patients had both APA and absent pulmonary valve.
Table 1 determines the characteristics of all the nine non-operated patients for whom we attempted to find and rehabilitate the concealed pulmonary arteries.
Group one patients, the successful group:
We described three of our patients for whom the DA finding and stenting to the hidden LPA were performed successfully.
The patient, one in Table 1, with arterial oxygen saturation of 55% in room air, was referred for further evaluation and possible DA stenting.
Catheterization revealed a good-sized right pulmonary artery from the right ventricle with no LPA opacification.
Aortography in anteroposterior view showed a blind-stump of the DA from the beginning of the left brachiocephalic artery towards the LPA.
A Hi-Torque Pilot coronary guidewire (Abbott Vascular) was steered from a right guiding catheter via a retrograde path through the stump of the DA to enter the LPA, and a non-compliant 2.5 × 15-mm balloon was inserted and repeatedly inflated as predilation at proximal and distal parts of the stenosis. Afterward, the aortography showed the LPA opacification from the stent.
Next, a bare 3.5 × 12-mm stent was inflated, and the result was promising (Figure 2).
Patients two and three in Table 1 underwent pulmonary artery CT angiography before the catheterization, which showed some small hypoplastic sparse artery branches in the left lung with no remnant of the LPA at the hilum that was non-suitable for surgical correction or palliation (Figure 3-b).
We performed a cardiac catheterization and right ventriculography in anterior-posterior and left anterior oblique views, which showed a good-sized right pulmonary artery but no LPA opacification.
Furthermore, ascending aorta injection showed the right aortic arch and one blind-ending stump of the DA possibly towards the left lung.
We retrogradely advanced one Asahi Fielder 0.014 guidewire from a right guiding catheter through the pouch and entered the LPA. After that, we repeatedly inflated a non-compliant 3 × 10-mm balloon into the stenosis; the aortic injection showed the fade staining of a small stenotic LPA from the DA.
Then, we successfully inflated a bare 4 × 18-mm stent into the DA in order to enlarge the LPA (Figure 3-c).
Figure 3-d shows the 3-dimensional CT angiography of patient number two, six months after the procedure with an acceptable condition for TOF total correction.
Group two patients, the suitable group:
Aortography was done for group 2 patients (Table 1), which showed a closed DA stump. We decided to stent the DA but at first, tried to do pulmonary vein wedge angiography to see the pulmonary artery retrogradely and to estimate the distance between the DA and the remnant of the LPA.
Luckily, the wedge angiography showed an LPA with a suitable diameter, and there was no need for rehabilitation with a stent or a surgical shunt; thus, we stopped the procedures (figure 4).
Group three patients, non-successful group:
Patients six and seven in Table 1 underwent CT angiography and aortography in multiple views, which did not reveal the LPA or remnant of the DA (Figure 5). The interatrial septa were intact, and pulmonary vein angiography was not performed due to the lack of guardian’s consent for atrial septostomy. If the foramen ovale had been open, the catheter could have been passed through the interatrial septum and we would have done pulmonary vein angiography to look the LPA situation.
On the other hand, patient number 8 in Table 1 had a remnant of the DA, and was a 5.5-year-old girl whose cardiac catheterization at 1.5 years of age had revealed absent LPA and a DA diverticulum; and, the total correction was carried out with right pulmonary artery and a homograft. However, she gradually developed pulmonary artery hypertension.
The previous aortic angiography was reviewed, which showed an acceptable-sized DA diverticulum, located underside of the aortic arch (Figure 6-a). Accordingly, we decided to open the stump to the closed DA with a coronary stent.
The right axillary artery approach was chosen to insert the head of the right guiding catheter exactly in the closed DA stump. Nevertheless, aortography showed that the stump was shorter than the previous angiography.
We could not steer 0.014 guide wires Asahi Fielder, and then Pilot Abbott Vascular from the DA stump to the LPA; the end of the pouch was completely closed, and it was not possible to cross the wire (Figure 6 b - c).
Patient 9 was similar to the 8th case, hence not described for brevity. If we had done the cardiac catheterization sooner, the wire might have been passed through the PDA toward the possibly existing LPA.
Our operated patients
We assessed our six surgically-corrected patients other than these nine, who were operated with one pulmonary artery using a homograft or Contegra from the right ventricle to the right pulmonary artery.
The mean age of them was seven years with a range of 6 to 9 years, and the mean follow-up time was four years.
All six cases were in NYHA functional class 2-3, and echocardiography revealed severe pulmonary artery hypertension. They also developed right ventricular dysfunction while the left ventricular functions in 2-dimensional echocardiography were normal (Table 2).
Table 2 Characteristics of the six TOF patients corrected with the absence of the left pulmonary artery
Patients
(No)
|
Sex
|
F/U
(year± SD)
|
TR PG
(mm Hg)
|
PS PG
(mm Hg)
|
TAPSE (mm)
and Z-score
|
EF%
|
SF%
|
M
|
F
|
6
|
2
|
4
|
4± 2.3
|
62
|
18
|
8
Z-score = -3.7
|
72
|
39
|
EF, ejection fraction; F, female; F/U, follow up; M, male; No, number; PG, pressure gradient; PS, pulmonary valvular or supravalvular stenosis; SD, standard deviation; SF, shortening fraction; TAPSE, tricuspid annular plane systolic excursion; TOF, tetralogy of Fallot; TR, tricuspid regurgitation