Ventricular septal defect (VSD) with pulmonary atresia (PA) can be considered to be
the severest form of tetrology of Fallot wherein the right ventricular outflow tract
obstruction has progressed to the extent of atresia. This atresia can occur either
at the infundibulum or as a plate atresia of the pulmonary valve. An important observation
is that the plate-type atresia is more frequently associated with well-developed pulmonary
arteries. The other significant abnormality in patients with VSD and pulmonary atresia
(PA) is the presence of arborization abnormalities. The blood supply to a particular
lung segment can be derived from a systemic artery or a central pulmonary artery or
a combination of both. These major aorto pulmonary collaterals (MAPCAS) expose a particular
lung segment to the systemic arterial pressure and produce local pulmonary vascular
changes akin to pulmonary arterial hypertension or, paradoxically, the MAPCA may develop
significant proximal stenosis over a period of time, and thereby, prevent the development
of local pulmonary vascular changes. It is important to identify the extent of structural
abnormality of the central pulmonary arteries, as these are crucial in planning the
management strategy. These may be confluent, nonconfluent or totally absent. When
present their size may be highly variable and hence of surgical significance.
SURGICAL APPROACHES
VSD with PA was first corrected by Lillehei[1] in 1955 using controlled cross-circulation
and a series of 10 cases was reported. Since then, the management strategy has evolved
considerably. Initially the popular approach was “staged” in which following an initial
surgical palliation to relieve hypoxia, a unifocalization procedure was done. This
was followed by the placement of a right ventricle (RV) to pulmonary artery conduit
and closure of the VSD. More recently, single stage unifocalization, VSD closure,
and RV to pulmonary artery conduit placement has become a popular approach with many
groups as illustrated in the accompanying paper by Murthy et al.[2] In those who can
not be successfully palliated by either of these approaches, heart lung transplant
remains the only option. However, it is extremely difficult to make generalizations
and the initial procedure of choice varies from patient to patient depending upon
the anatomy of central pulmonary artery, arborization abnormality, age of the patient
etc. The accompanying paper has already discussed the single-stage approach in great
detail, so, it may be pertinent here to discuss the staged approach.
The aims of the staged approach in a patient with VSD, PA and MAPCAs are (a) to increase
the central pulmonary artery flow by establishing a direct continuity between the
ascending aorta or the RV and the small pulmonary artery thereby stimulating its growth:
Stage I (b) unifocalization of MAPCAS in both lungs: Stage II, and (c) closure of
the VSD and establishment of RV to pulmonary artery continuity: Stage III. The principle
behind staged procedure is that even very small native central pulmonary arteries,
have a potential to grow. Therefore, augmentation of blood flow to the central pulmonary
arteries may lead to gradual and better “rehabilitation.” The main advantage of the
staged approach is that it breaks the entire procedure into less stressful and better-tolerated
smaller surgical segments. Additionally, management of MAPCAS may be much easier by
via a posterolateral thoracotomy than through a sternotomy approach. The palliative
procedures have the potential to increase the pulmonary blood flow and promote growth
of even diffusely very small right and left pulmonary arteries, which may otherwise
not be amenable to direct surgical enlargement.[3] The systemic to pulmonary artery
shunt (most often a Blalock–Taussig shunt) enlarges the ipsilateral pulmonary artery
to the same extent as the contralateral pulmonary artery.[4] In the presence of short
segment PA, an option is to place a transannular patch across the right ventricular
outflow tract and in-to the main pulmonary artery. This involves incising the main
pulmonary artery longitudinally and extending the incision across the pulmonary annulus
into the right ventricular outflow tract. The right ventricular to pulmonary artery
continuity is then established by autologous pericardium or low porosity Dacron.[5]
Although this procedure is beneficial clinically as evidenced by improved arterial
oxygen saturation and decreased hemoglobin values, it has a high incidence of producing
stenosis either in the right or the left or both the pulmonary arteries.[6]
Another option is central ascending aorta to main pulmonary artery shunt,[7] (Melbourne
shunt Figure 1), which is considered in the presence of confluent hypoplastic pulmonary
arteries. In the presence of a tapering main pulmonary artery, it can be detached
from the right ventricular outflow tract and anastamosed to the ascending aorta. The
central shunts promote a more uniform growth of the pulmonary arteries. However, they
may lead to early development of congestive heart failure and pulmonary arterial hypertension
and hence the patients need to be followed closely.
Figure 1
Melbourne shunt: side-biting clamp controls the ascending aorta; soft clamps control
the branch pulmonary arteries. Inset demonstrates the completed shunt with the pulmonary
artery anastomosed to the posterior and left lateral aspect of the ascending aorta
close to the sinotubular junction. Reproduced with permission from Duncan et al.7
The second stage involves MAPCA ligation and transplantation. Ligation is done when
there is dual blood supply to the same segment of lung from native pulmonary artery
as well as from the MAPCA. MAPCAs need transplantation when they are the only source
of blood supply to a broncopulmonary segment and there is no peripheral stenosis and
they are not hypertensive.[5]
The MAPCAS can be directly anastamosed end to side with branch pulmonary artery or
one MAPCA is anastamosed to the branch pulmonary artery and the others are anastamosed
to this MAPCA. Azygos vein has also been used as an interposition graft to transplant
MAPCAS.[8]
This approach of staged unifocalization allows the surgeon to tailor the surgical
procedure to the specific needs of every patient depending on the existing anatomy
and physiology.[7] For patients who have congestive heart failure, unifocalization
is initially performed on the side with the least obstructed pulmonary blood flow,
which makes congestive heart failure easier to manage and decreases the likelihood
of development of obstructive disease in overcirculated pulmonary segments. Patients
with significant cyanosis have unifocalization performed initially on the side with
the most obstructed MAPCAs. Modified Blalock–Taussig shunt is often performed adjunctively
at the time of unifocalization in these cases to further augment pulmonary blood flow
with a resulting decrease in cyanosis. Staged unifocalization may not always require
bilateral thoracotomies in addition to median sternotomy. Large central MAPCAs that
originate relatively close to the central pulmonary arteries (especially those on
the left side) may be easily unifocalized at the time of complete repair through a
median sternotomy. In the majority of cases all unifocalization and ultimate complete
repair can be performed within a year after entering the operative sequence that commences
with the performance of a central shunt.
The last stage involves closure of ventricular septal defect and establishment of
continuity between the right ventricular outflow tract and pulmonary artery. The Birmingham
formula is used preoperatively to calculate the postoperative ratio of the right ventricular
to left ventricular pressure (pRV/LV) ratio and patients with pRV/LV ratio < 0.7 are
considered suitable for repair.[5]
In a landmark paper on the staged approach,[5] Drs Iyer and Mee reported the algorithm
and results of their approach in a series of 58 consecutive patients over a 10-year
period. A total of 121 staging procedures were performed with an overall mortality
of 10.3%. One hundred thirty-four major collaterals were either ligated or transplanted.
Thirty patients eventually underwent complete repair with an early mortality of 3.3%
and late mortality of 10.0%. Twenty-six current survivors of repair remained clinically
well after a mean follow-up of 3.6 years. Ten patients were in various stages of preparation.
Twelve patients (20.7%) failed to achieve minimum requirements for repair after staging
and were awaiting further palliation or heart–lung transplantation when this study
was published.
In a large series from Los Angles[9] involving 104 patients undergoing staged repair,
58 patients (55.765) achieved complete anatomic repair. The mortality in stage 1 repair
was 6%, 9% in stage 2 and 8.5% in stage 3. The 10-year mortality was 16.5%. The median
pRV/LV was 0.5. The number of collateral vessels incorporated in the repair was an
independent risk factor for postoperative mortality and an elevated pRV/LV. The authors
had a simple management algorithm for these patients, which is presented in Figure
2.
Figure 2
Management algorithm for patients with pulmonary atresia with ventricular septal defect
(VSD) and major aortopulmonary collateral arteries (MAPCAs) based on the nature of
pulmonary vascular supply. Reproduced with permission from Gupta et al.9
Recently, the group from Shanghai, China has reported a different two-stage approach.[10]
In the first stage, a left thoracotomy is performed along with unifocalization of
the left-sided MAPCAs into a vascular graft. A systemic to this vascular graft shunt
is added and the other end of the graft is closed to form a caecum, which is then
placed under the aortic arch so that it can be approached at the next stage through
the midline. At the next stage, 6 to 11 months later, a median sternotomy is performed
and the unifocalization of the right sided MAPCAs is achieved into another vascular
graft. The previous left shunt is taken down and after establishing cardiopulmonary
bypass, the two vascular grafts: right and left are connected to each other and a
RV to this graft confluence conduit is placed along with VSD closure. Using this approach,
the authors did not have any mortality at the first stage and there was only one death
in the 11 patients undergoing completion of the repair at the second stage. There
were no late deaths and no reintervention during the mean follow up period of 25.4±15.2
months.
In another report from France, Metras et al. adopted a different staged procedure
in patients with extreme hypoplasia of the pulmonary arteries.[11] The initial stage
(performed as early as 10 days, range 0.1–18 months) involved rehabilitation of pulmonary
arteries by direct continuity between ascending aorta or right ventricle and the diminutive
pulmonary arteries, followed by interventional catheterization (stenosis dilatation,
pulmonary artery stents and coil occlusion of MAPCAS) and a subsequent complete correction
with closure of ventricular septal defect and right ventricle to pulmonary artery
conduit. There was 90% survival after the first stage. Seventy percent patients had
complete correction. During the follow up of 83±65 months, all patients had improved,
50% had no cardiac medications, none had residual shunt, RV/LV pressure ratio was
0.6 (range 0.3–1). In this series in all cases, the main pulmonary artery branch size
was between 1 and 2.7 mm (mean 1.45 mm) and the Nakata index was 3.5–58, mean 20.6
mm2/m2indicating extreme hypoplasia of the pulmonary arteries. At the second stage,
there was satisfactory growth of these pulmonary arteries [Figure 3].
Figure 3
(a) Extremely diminutive central pulmonary arteries (Nakata: 15) showing the ‘‘sea-gull’’
aspect, filled by a retrograde angiogram in a pulmonary vein. A MAPCA is also opacified
retrogradely. (b) Three months after RV–PA connection by patch done at 4 months of
age, there is a nice development of the PAs, with normal pressures and satisfactory
distribution. Reproduced with permission from Metras et al.11
Having summarized the various approaches to the management of VSD, PA, and MAPCAs,
one should not forget that there is extreme variation in the anatomy, which will require
individualization of the approach to any given patient. At the same time as detailed
by Murthy et al., in the accompanying paper the single stage approach still has the
advantages of preventing development of stenosis in MAPCAS and any pulmonary hypertensive
changes, and because it is performed early in life, both MAPCA stenosis and pulmonary
arterial hypertension are unlikely to develop by this age. The other advantage of
single staged repair is early normalization of physiology and correction of polycythemia
and cyanosis during infancy.[12
13] It also avoids long-term cardiac dysfunction due to prolonged cyanosis and arrythmias.
However, the superiority of the single over the multiple stage approach will require
long-term, prospective, randomized, multicentric trial, which seems to be distant
as of now.