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Fig.1 (Type A, B, C): Classification of PA - VSD according to the status of native pulmonary arteries (NPAs), aorto-pulmonary
collaterals (APCs) and patent ductus arteriosus (PDA).
Type A: Native pulmonary arteries present, no APCs.
Type B: Native pulmonary arteries and APCs present.
Type C: No native pulmonary arteries, only APCs maintain pulmonary blood flow.
arteries are present. In PA-VSD, PDA typically age. Color Doppler flow studies have been
originates from either the undersurface of the shown to be reliable in making this distinction
arch (67%) or from the undersurface of the between PDA and APCs based on the direction
innominate artery (33%). Unilateral PDA is of blood flow in the proximal mediastinal
usually associated with confluent PAs, while PDA segment of PAs6. Furthermore, PDA is straight
can be bilateral as is usual with non-confluent and do not branch while APCs in general, are
PAs. When PDA is present, PAs are confluent in tortuous and may branch.
80% of cases. All patients with PDA have central
PAs6. Notably, PDA is absent in 1/3 of cases and V. Classification
is associated with absent central PAs6.
Aortopulmonary collaterals (APCs) are The anatomic spectrum varies from atresia of
muscular arteries until they enter the lung pulmonary valve, presence of MPA and
parenchyma, but the muscular layer is gradually confluent normal sized PAs that are supplied by
replaced by elastic lamina that resembles true a PDA; to atresia of MPA with diminutive and/or
pulmonary arteries. APCs are seen in 30 – 65% non-confluent PAs, absent PDA and pulmonary
of patients with PA - VSD7 and are usually 2 – 6 blood supply solely provided by multiple APCs
in number. Known sites of origin of APCs include and bronchial arteries. There are several
descending thoracic aorta at the level of carina, degrees of severity in between these two
subclavian arteries, abdominal aorta, and extremes of the spectrum. Consequently, it has
coronary arteries. Sixty percent of APCs have been difficult to classify this lesion and compare
stenosis either at diagnosis or it develops over a the outcome. A practical classification has been
period of time during follow up. proposed by Congenital Heart Surgeons Society
The differentiation between PDA and APCs is based on complexity of pulmonary blood supply
important in newborns, who have balanced which in turn indicates the complexity of surgical
pulmonary blood flow and therefore, are repair1 (Figure 1).
candidates for a relatively late definitive repair. In Type A: Native PAs present, pulmonary
such patients, a reliable source of pulmonary vascular supply through PDA and no APCs.
blood flow is necessary until cardiac repair is Type B: Native PAs and APCs present
performed. PDA is considered a less reliable Type C: No native PAs, pulmonary blood
source beyond the first few days of life due to its supply through APCs only.
tendency to close. Though APCs are also prone Surgical approach for type B and C is similar
for stenosis over a period of weeks to months, except that more extensive unifocalization of
they remain patent more reliably than PDA until APCs will be needed in Type C, before the total
surgical repair is performed at few months of repair is achieved.
VI. Evaluation of a child with PA-VSD the newborn gets older and leads to heart failure.
These infants may have only mild cyanosis and
VI.1: Clinical presentation escape recognition until later.
There is yet another subset of patients with
Approximately 65% of Tet-PA patients present to adequate and “balanced” pulmonary blood flow
a cardiac center during infancy. The remainder throughout early infancy and may present during
presents later presumably because of high late infancy. Such patients may present with a
enough pulmonary blood flow which lead to heart murmur that was heard during routine
clinically undetectable cyanosis during the early physical examination and cyanosis or heart
months of life. Overall, the modes of failure was not clinically obvious. As a general
presentation in Tet-PA consisted of cyanosis rule, in complete mixing lesions such as
(50%), heart failure (25%) or murmur with mild pulmonary atresia, systemic oxygen saturation
cyanosis with or without failure to thrive (25%)5. of 85% is achieved by Qp/Qs of at least 2.5
Newborns present with cyanosis with or (mixed venous saturation 60%). Symptoms of
without a heart murmur. Such newborns have heart failure in childhood imply a Qp/Qs ≥ 4. The
duct dependent pulmonary circulation. The so-called “balanced circulation” with
presentation occurs when the duct starts to asymptomatic infants occurs when Qp/Qs
constrict. Severe hypoxia, acidosis and shock ranges between 2.5 and 4 during infancy5.
ensues closure of PDA. If the babies had gone Failure to thrive in the absence of heart failure
home by this time, they present to the has been reported as a presenting symptom but
emergency department in extreme shock and the mechanism is unclear and can be secondary
acidosis. Typical age of presentation in this to underlying genetic abnormality.
group is 3 – 7 days. Sepsis, congenital adrenal Adult patients, either unoperated since they
hyperplasia, other duct-dependent congenital were deemed inoperable or had undergone only
heart diseases or severe illnesses affecting a palliative procedure, are infrequently seen in
other systems comprise the differential the current era. In a recent report of 26 adult
diagnosis. patients4, all were cyanotic (mean oxygen
There may or may not be a murmur which is saturation of 85%) and polycythemic (mean
typically continuous in nature representing hematocrit 57%) at presentation. They were all
aortopulmonary collateral artery flow if present. symptomatic with signs of heart failure such as
Immediate resuscitation with prostaglandin E1 effort dyspnea or decreased exercise tolerance
(PGE1) infusion will help to stabilize the patient. and were NYHA functional class II or III.
This is the type with good-sized native PAs
which are supplied by a duct. Usually, these VI.2: Physical examination
patients do not require unifocalization and are
good candidates for neonatal repair with right The severity of cyanosis depends upon the
ventricle to pulmonary artery (RV-PA) conduit. amount of pulmonary blood flow. Close clinical
However, babies with more complex pulmonary follow up with regular measurement of oxygen
blood flow tend to be less dependent on ductal saturations is essential until surgical repair is
flow since the proportion of pulmonary blood flow accomplished. On the other hand, fall of
derived via native PAs is much less than that pulmonary vascular resistance during early
derived via the APCs. If the pulmonary blood infancy allows increase in pulmonary blood flow
flow is adequate and well-balanced, these leading to heart failure and present with feeding
babies will only have mild cyanosis and will difficulty, failure to thrive, signs of respiratory
escape detection as a newborn. distress, tachypnea, tachycardia and
Presentation in early infancy occurs when the hepatomegaly. A bounding pulse in these infants
baby has “balanced circulation” with adequate is usual and signified large pulmonary blood flow
pulmonary blood flow via APCs. These babies with run-off from systemic arteries through
often present after 4 – 6 weeks of age either with APCs. Auscultation reveals the absence of
increasing cyanosis or signs of heart failure. pulmonary component of second heart sound
Development of stenosis in APCs progressively and therefore, a single S2. Continuous bruit of
reduces pulmonary blood flow causing the flow through APCs could be heard over the
progressive cyanosis. Alternatively, pulmonary chest wall. As the infancy progresses, cyanosis
over circulation occurs from the physiologic usually worsens and polycythemia and clubbing
reduction in pulmonary vascular resistance as may develop.
Fig.2 (A, B): PA-VSD with confluent native pulmonary arteries (NPAs) and aortopulmonay collaterals (APCs).
A: Aortic arch angiography by pigtail in anteroposterior (AP) view showing confluent NPAs and APCs.
B: Selective left lung collateral angiography using Judkins right catheter in AP view showing retrograde filling of NPAs via the
APC that originates from descending thoracic aorta.
pulmonary artery index for use in patients with vessels only. The nature of the distal pulmonary
such complex lesions. vascular bed and pulmonary vascular resistance
3) Total Neo-pulmonary artery index (TNPAI): are not expressed in these calculations. Since
Nakata index is of limited use for evaluation of these latter factors play an important role in
the adequacy of PAs in single stage repair postoperative RV pressure and in turn the
strategy where unifocalization of several APCs is hemodynamic outcome of surgical repair, an
followed by total repair at the same operation. In intraoperative method to assess the adequacy of
Nakata index, there is no provision for the pulmonary vascular bed has been proposed15.
additional vascular bed that will be added by
unifocalization. A composite index of native PAs VII. Management
and the APCs that will be unifocalized was
needed, in order to determine whether the VSD VII.1: General principles of surgical therapy of
could be closed at surgery. PA-VSD: Heterogeneity of pulmonary blood
The UCSF group proposed TNPAI in order to supply in PA-VSD precludes uniformally
help preoperative planning in these patients15. applicable management to all the patients.
Nakata PA index was measured as described However, certain guiding principles of
above. Then, APCs index was calculated by management have evolved over the past 3
addition of CSA of all significant APCs divided by decades based on earlier observations in these
the BSA. CSA of each APC was calculated from patients. Connecting as many lung segments as
diameter of the respective vessels measured on possible to the blood flow from RV during early
preoperative cineangiogram. The sum of total infancy is essential since early attrition of these
APC index and PA index is called TNPAI. A patients occurs during infancy and significant
TNPAI index >200 mm2/m2 correlated well with histologic changes occurs in pulmonary
low postoperative RV/LV pressure ratio and vasculature during young age5,9. Development of
identified patients who were clear candidates for pulmonary vascular occlusive disease from
VSD closure at the time of single-stage surgical unrestricted pulmonary blood flow from APCs
repair. These indices are limited in value since can develop as early as 4 weeks16. Recruitment
they are based on the size of the proximal of lung segments into RV-PA conduit supply is
more successful when blood flow to it is restored
early in life and complete repair should be
attempted within weeks to months during
infancy. Therapeutic catheterization procedures
such as balloon angioplasty help to rehabilitate
pulmonary arteries with stenosis and should be
combined with surgical repairs to optimize the
overall outcome.
VII.2: Components of surgical repair:
Regardless of the surgical strategy that is used
for a given patient, the components of total repair
of PA-VSD consist of (a) placement of RV - PA
conduit, (b) unifocalization of APCs and (c) VSD
closure. These components are performed in
one-stage, or at different operations depending
on the anatomy and institutional policy.
a) RV – PA conduit placement: Typically a
cadaveric, cryopreserved homograft is used to
connect right ventricle to available central
pulmonary arteries. In complex cases, where a
central pulmonary artery is absent or the
pulmonary blood flow is multifocal,
unifocalization of the diminutive native
pulmonary arteries and APCs will be performed
Fig.3: Cartoon showing repaired Pulmonary atresia - before RV – PA conduit is placed (Figure 3).
Ventricular septal defect. Right ventricle to pulmonary
artery (RV - PA) conduit is shown, VSD patch not shown in b) Unifocalization of APCs: It was shown in
the cartoon. the mid seventies that unifocalization will enable
connecting more lung segments to central when applicable. When VSD closure is deferred
Pas17,18, and the current practice is to unifocalize at initial repair, it is surgically closed after 6 - 12
significant APCs during the first 3 months of life. months, if and when left to right shunt is
Median sternotomy is the preferred method established via the VSD with Qp/Qs exceeding
especially if single stage repair is planned. In 2:1 by catheter evaluation15.
multi stage surgical approach, unifocalization is
done through lateral thoracotomies. During VIII. Multi-stage versus single-stage
unifocalization, APCs are ligated at the origin approach
and mobilized to maximize their length with
creative rerouting. Such mobilized vessels are VIII.1: Multi-stage approach: A multi-stage
anastomosed in the mediastinum before being correction evolved from the early surgical
connected to RV-PA conduit. experiences. Inevitably, the strategy changed
c) VSD closure: Closure of VSD at the time of based on individual patient's anatomy and clinical
initial repair is desirable in order to avoid the need features. Traditional approach consisted of a
for further surgery. However, if there were any palliative shunt in all patients (patients with “good
concerns about the adequacy of the pulmonary size”, confluent central PA in particular) during
vascular bed, it is customary to defer VSD neonatal period or early infancy to relieve
closure. Leaving the VSD unrepaired, helps to cyanosis and allow for growth of distal pulmonary
avoid supra-systemic RV pressure in the arteries. However, with diminutive PAs, RV – PA
immediate postoperative period by allowing RV to continuity is established by placing a RV – PA
decompress through the VSD. Over a period of conduit. This provides catheter access to
months, pulmonary vascular development occurs peripheral PAs to perform balloon angioplasty of
and the VSD can be closed safely with sub- the pulmonary arteries. The VSD is typically left
systemic RV pressure. The strategy of delayed open at this first stage. Any possible
VSD closure has reduced the operative mortality. unifocalization of APCs will also be performed.
With the single stage surgical repair strategy it A subsequent operation will be done to close
is important to ensure that pulmonary the VSD, relieve any residual right ventricular
vasculature is adequate, both in diameter of outflow tract obstruction and place a valved
proximal pulmonary vessels and development of conduit. With absent mediastinal PAs, the
distal pulmonary vascular bed, for the safe surgical approach is further complicated. Two
closure of VSD. Preoperative PA indices modified Blalock Taussig shunts are performed
mentioned earlier help to assess the adequacy to each PA via bilateral thoracotomies.
of PA size and the nature of distal pulmonary Unifocalization of any significant APCs will be
vascular bed that is connected to central PAs. preformed. Each thoracotomy is done during the
However when a single-stage repair strategy same hospitalization but separated by few days.
is adopted with unifocalization of APCs at the This will relieve cyanosis and allow growth of
same operation, preoperatively-determined PA native pulmonary arteries. The babies would
indices will not be able to predict the level of have catheter evaluation prior to next operation.
pulmonary vascular bed added by unifocalization The second operation will consist of RV – PA
of APCs. Therefore, an intraoperative method to homograft, connection of all branches of PA with
evaluate adequacy of pulmonary vascular bed or without VSD closure. Modifications to above
was proposed by the UCSF group15. After mentioned generalized outlines will be made
completion of unifocalization and distal dependent upon individual patient's condition.
anastamosis of RV - PA conduit, a perfusion VIII.2: Single-stage approach: Current
cannula and a PA catheter are inserted from the surgical approach attempts to perform APCs
proximal end of the conduit and left atrial vent is unifocalization and cardiac repair at the same
placed. The conduit is connected to the bypass operation, through median sternotomy. The
machine. The bypass machine is run at choice between multi-stage and single-stage
increasing flow rates to 2.5 L/min/m2 and the PA repair is dependent on various factors: Nature of
pressure is monitored. VSD is closed if the mean PAs (small vs. good size), (duct-dependent or
conduit pressure is < 25 mmHg, and left open if collateral-dependent PBF), age of the patient at
it is higher. Alternative strategy in borderline presentation, status of APCs, and availability of
cases is to close the VSD with a fenestrated surgical skills and results of the institution.
patch and the fenestration can be closed later Newborns with no PDA and adequate collateral
either by surgery or transcatheter technique, dependent pulmonary blood supply with
acceptable systemic oxygen saturations, are the Aortic regurgitation (AR) develops in a significant
typical candidates for elective single-stage number of patients with or without complete
unifocalization and cardiac repair that is surgical repair. Development of AR occurs more
performed at about 3 months of age. often with patients who had palliative shunts only
VIII.3: Comparison of outcome between multi since they add to the LV volume overload and
and single-stage repair: Several theoretical therefore LV dilatation. The resultant aortic
advantages of the single-stage approach over the annular dilatation worsens aortic regurgitation.
more traditional multi-stage approach exist. Single Infective endocarditis affecting aortic valve is
stage repair allows for early normalization of another mechanism of AR. Progressive LV
cardiovascular physiology by recruitment of all dilatation due to volume overload from AR,
possible lung segments into RV derived circulation systemic to pulmonary artery shunt or collateral
as early in life as possible. This alleviates cyanosis flow eventually leads to LV dysfunction.
and polycythemia during infancy. Early repair also In patients who have had complete repair, there
preserves pulmonary vascular bed and avoids is a gradual deterioration of conduit function23 from
development of pulmonary vaso-occlusive disease loss of luminal diameter, calcification, peel
in the lung segments exposed to systemic formation and from the deterioration of valve
pressure via APCs, and hypoplasia of the distal function. The valve in the conduit is prone for
pulmonary vasculature in under-perfused lung calcification, stenosis and regurgitation.
segments. There is also evidence to suggest that Pulmonary regurgitation worsens with any residual
long term cardiac function is preserved by avoiding stenosis in distal pulmonary arteries. While
ventricular dysfunction from prolonged cyanosis pulmonary regurgitation is well tolerated for years,
and arrhythmias19. When we compare outcome RV dilatation and hypertrophy eventually ensues
between patients treated in same surgical era20, 21, leading to RV dysfunction24. There is evidence that
the ultimate results are comparable but patients in RV dilatation with dysfunction can eventually
the single stage group undergo one or two impact LV function by ventricle-ventricle
operations less than the patients in multi-stage interaction. However, optimal timing of re-
group do. operation either to replace the deteriorated conduit
or implantation of pulmonary valve to stop
IX. Complementary role of pulmonary regurgitation is still unclear.
interventional catheterization Development of ventricular arrhythmias has been
documented after tetralogy repair. This is thought
Interventional catheterization has assumed an to account for the relatively high incidence of
important complementary role in rehabilitation of sudden deaths noted in patients long after
pulmonary arteries in the management of tetralogy repair. Co-existing poor hemodynamic
patients with PA - VSD by the use of balloon parameters such as high RV pressure is thought to
angioplasty and stent placements 22. be a risk factor for arrhythmias. Correction of
Catheterization helped avoiding surgery in case hemodynamic abnormalities by pulmonary valve
of stenosis in proximal segments of the PAs and implantation or replacement of RV – PA conduit is
by being able to reach distal stenosis within lung expected to help reduce this risk25. The outcome
parenchyma that are inaccessible to the from the current modified approach combining
surgeon. Coil occlusion of APCs, stent surgery and therapeutic cardiac catheterization
placement in RVOT and palliative stenting of techniques has improved the outcome and long
stenotic APCs are some of the other procedures term studies in future will provide proof of such
that interventional catheterization has to offer to improved outcome.♦
the patients with PA – VSD.
References:
X. Long term sequelae/outcome
1. Tchervenkov CI, Roy N. Congenital heart
Many of the long term sequelae have been disease nomenclature and database project:
mentioned earlier under the natural history Pulmonary atresia - ventricular septal defect. Ann
section. Patients who were unsuitable for Thoracic Surg 2000; 69:S97-S105.
complete surgical repair and therefore were 2. McElhinney DB; Reddy MV; Hanley FL.
palliated with systemic to pulmonary artery Tetralogy of Fallot with major aortopulmonary
shunts only, develop progressive cyanosis and collaterals: Early total repair. Pediatric Cardiology
polycythemia as they survive into adulthood. 1998;19:289-296.