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Arq Bras Cardiol. 2021; 117(4):664-665
Short Editorial
Transcatheter Palliation for Tetralogy of Fallot
Francisco Chamié1
Intercat - Cardiologia Intervencionista,1 Botafogo, RJ - Brazil
Short Editorial related to the article: Palliative Endovascular Intervention in Infants with Tetralogy of Fallot: A Case Series
Mailing Address: Francisco Chamié •
R. Ministro Armando de Alencar, 16/201. Postal Code 22.471-080,
Lagoa, RJ – Brazil
E-mail: chamief@gmail.com
Keywords
Tetralogy of Fallot; Cyanosis; Cardiac Catheterization; Stents.
The Tetralogy of Fallot (TOF) is the most common cyanotic
congenital heart defect. The morphologic key feature of TOF is
the malalignment of the infundibular septum. The infundibular
septum malalignment makes the aorta override interventricular
septum (dextroposition) over a large ventricular septal defect
(VSD) and promotes obstruction to the right ventricular
infundibular tract. The pulmonary valve is also stenotic, and the
pulmonary trunk and arteries are, to some degree, hypoplastic.
Severe obstruction to pulmonary blood flow (PBF) leads to more
systemic unsaturation and, thus, prolonged hypoxia.
Open heart surgery is the traditional treatment modality,
augmenting the right ventricular outflow tract (RVOT), closing
the VSD, thus redirecting the aorta to the left ventricle,
correcting the cardiac anatomy, and normalizing systemic
flow saturation.1
Some patients are not candidates for early surgery due to
insufficient body weight, small pulmonary artery size (bad
anatomy), prematurity, neurologic impairment, or associated
defects.2,3 In those cases, palliative procedures are necessary
to increase PBF and systemic oxygen saturation, reducing
levels of hypoxia. Ideal palliation would offer a stable and
symmetric pulmonary blood flow source and adequate growth
of pulmonary arteries, leaving behind no residues that can
impair corrective surgery.
The most traditional form of palliation is the Blalock-Taussig
Shunt (BTS), idealized by Alfred Blalock and Helen Taussig and
first performed in 1944 by Alfred Blalock.4 Classic BTS consists of
right subclavian artery anastomosis to the right pulmonary artery
when the aortic arch is left-sided. When the aorta is right-sided,
the anastomosis is performed in the left subclavian artery.
Modified BTS using interposition of a PTFE tube graft was
later developed in the ‘70s aiming to preserve subclavian flow
to the ipsilateral arm.5 Although effective, BTS has some issues,
including selective PBF, unequal development of pulmonary
arteries, suture-mediated pulmonary branch stenosis, and
shunt occlusion with consequent hypoxemia. In addition, it
should be considered that transporting a severely ill patient
to the operating room is a risk in itself.
Non-surgical palliation alternatives were pursued, and
several strategies were offered, such as pulmonary balloon
valvuloplasty (PBV) and ductal or RVOT stenting.
PBV is effective in cases where the main obstructive
feature is the pulmonary valve, having reduced efficacy
when significant infundibular stenosis is present. In that case,
effective palliation has short-term efficacy.6
Ductal stenting is a safe and effective procedure when
performed in experienced centers. It has comparable results to
BTS in selected patients with ductal-dependent PBF. Glatz et al.
report on 106 patients treated with ductal stenting versus 251
patients treated with BTS. Primary composite outcomes (death
or reintervention) were more common in the BTS group (29.5%
vs 17%, p= 0.014) due mainly to unplanned reinterventions for
cyanosis relief (10.4% x 6.6%, p=0.26). As anticipated, procedural
complications were more commonly found in the BTS group,
although without statistical significance. Pulmonary artery growth
in the ductus stenting group was greater and more symmetric
(p=0.015).7 Possible complications of ductal stenting are in-stent
re-stenosis, intimal proliferation, and stent obstruction.8
RVOT stenting emerged as a compelling technique for TOF
palliation. Relief of infundibular obstruction and pulmonary valve
stenosis by bare-metal stent implantation in the RVOT can lead
to stable PBF and satisfactory growth of pulmonary arteries.9-13
RVOT stenting improves the pulsatile flow of systemic
venous blood to the pulmonary artery, improving oxygen
saturation without a decline in diastolic aortic pressure
and resultant coronary perfusion. A systematic review and
meta-analysis by Ghaderian et al. showed high clinical efficacy
of RVOT stenting in achieving adequate pulmonary arterial
growth during palliation and obtaining proper arterial oxygen
saturation. They also showed low post-procedure morbidity and
mortality following RVOT stenting and no significant difference
in procedural outcomes.14 In small patients and complex
anatomies, RVOT stenting allows for surgical correction in a later
stage. Stent removal during surgery, albeit feasible, lengthens by-
pass time and, in most cases, determines the use of transannular
patches at the time of definitive surgery.15
In the current issue of Arquivos Brasileiros de Cardiologia,
Kupas et al.16 reported on six infants treated by RVOT stenting.
The median age at the moment of the implant was 146.5 days
and 367 days at the moment of stent implant and retrieval,
respectively. Four patients had an infundibular obstruction,
and two patients predominantly had a valvar obstruction.
The immediate post-procedure evaluation showed a reduction
of the peak systolic gradient, increased pulmonary arteries size
and systemic oxygen saturation. Overall mortality was 33%.
Thus, albeit constituting a very small case series, the authors
propose stent implantation in RVOT as an interesting and
appealing option for TOF palliation in very sick neonates.16
Transcatheter palliation may direct high-risk patients to
the path for complete and physiologic resolution of TOF.
New techniques on the horizon make TOF treatment very
likely to be performed less invasively, with catheter-based
and/or hybrid procedures.17
DOI: https://doi.org/10.36660/abc.20210735
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Arq Bras Cardiol. 2021; 117(4):664-665
Short Editorial
Chamié
Transcatheter Palliation for Tetralogy of Fallot
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