Dr Kiran welcomes everyone back to the blog of Pediatric cardiology department of Narayana Hrudayalaya, after a very long hiatus.
It has been more than two years and I often
wondered why am I not blogging anymore! As often teased, I had convinced myself
that I am the sole writer and reader of my blog! Also, more approachable media
like FB had come up with good forums for the discussions I had in my blog, with
much better responses. May be, I felt the effort of blogging was not worth the
sweat (and finger-pain) that went in making it.
But surprisingly, many had remembered the
blog! I was asked about it in clinical meetings, emails, FB and rarely over
phone also. I was given an understanding on how Google chooses it search
priorities in vain attempt to convince me to continue blogging! All said and
done, the inertia is lot difficult to break! Finally, here I am.
For a while, I will retain the old fashion!
If this attempt to continue the blog survives, then I have plans to review one
journal article on Pediatric cardiology every post, along with interesting
learning scenarios with some snippets if possible.
OBSTRUCTION FOR RESCUE
Preparation of LV in cases of dTGA with intact IVS is the
key for survival for those children who present late. Presence of VSD or large
PDA, LVOT obstruction, PPHN are some of the causes for LV preparation we have observed. We had an
interesting learning recently on this.
One-year-old baby presented to our OPD with
cyanosis. During echo, we found ambiguous situs with TGA, intact IVS with
moderate ASD. There was also obstructive cor-triatriatum for this child! The
ASD was distal to the obstruction. Jet from cor-triatriatum was directed to RA
across the ASD. The complex picture had eventually resulted in severe PAH which
had kept the LV
prepared! Added to this was L-posed great arteries, an uncommon association in
isolated TGA. More surprisingly, the arch was right sided, making the entire
combination truly unique. I would like to know the experience of the readers on
different ways of LV
preparation they have come across in TGA with intact IVS. Please let us know
your experience.
DIFFERENT CURVES
As an unspoken rule, we see left arch in
cases of L-malposed great arteries with any pathology underneath. How common is
the right arch in any case of L-malposed great arteries? What problems can be
anticipated in distal arch with this combination? We came across about three
such cases in last two years. None of these cases posed any significant
problems distal to arch. Please share you experience on this unusual
combination. Any embryological explanations for the same?
NOMENCLATURE
By definition, the ventricle should have at
least 50% of the corresponding AV valve committed to it. So, in cases of DILV,
the other chamber is called by different names: Rudimentary chamber, trabecular
pouch or outlet chamber depending on whether it is linked with only inflow,
only bulboventricular foramen or only outflow. Sometimes, we see this
unipartite chamber giving rise to both great arteries in DILV, which is called
DILV with DORV!! The question is, if this is not a ventricle by definition,
then how can we use the term DORV?! In that case, should we use a term or
should we simply elaborate the description? Please share your point of view and
what you do in your practice for such combinations.
SMOTHERING
Constrictive pericarditis is a gruesome
condition. In the third-world, tuberculosis is still the dominating etiology
for this, followed by septic infective etiology. Sometimes, we can see other
causes too, like connective tissue disorders. Recently we came across a
9-year-old girl with a rare genodermatoses called H-syndrome with association of cutaneous hyperpigmented, hypertrichotic, and
indurated patches associated with hearing loss, short stature, cardiac
anomalies, hepatosplenomegaly and hypogonadism. Her heart was structurally
normal but with effusive constriction. Her co-morbidities did not allow us to
be aggressive on pericardiectomy, which we felt was definitely beneficial. Has
anyone come across this syndrome earlier? What
cardiac findings did you observe? How did you go about? In general, how many of
us are really aggressive with cardiac intervention in presence of serious
co-morbidities?
COLLATERAL
TO PULMONARY ARTERY
In cases
of pulmonary atresia, AP collateral feeders to pulmonary artery are a common
finding. How about a large collateral forming one of the pulmonary artery? We
had a 4-year-old child with minimal cyanosis with no audible murmurs. Echo
showed pulmonary atresia with a large VSD with impressive pulmonary venous
return to LV,
causing LA/LV dilatation! There was no MPA. PDA from right arch was continuing
as RPA. LPA was not seen on echo, but the return from left pulmonary veins was
good. In pursuit of collaterals, we felt that we found double arch! However,
the right arch was giving rise to all 3 neck vessels along with PDA! On careful
chasing, the large vessel which we thought was a double arch could be traced to
bracheocephalic artery and tracing it distally, we lost it after it took a
deviation towards left. CT scan revealed this large collateral to be continuing
as LPA into the hilum of left lung. We still have difference of opinion on
whether to call it LPA or collateral to left lung! Our surgeons are working on
possibility of unifocalization. I shall keep the readers updated. It would be
interesting if readers can share their experience.
With
that, we come to the end of this post. Please pen in your comments. If you find
any problem in posting comments, please feel free to mail it to my emial id
I shall post them on your behalf.
Regards
Kiran
