Wednesday, November 27, 2013

“Usually, there is nothing more pleasing than returning to a place where you have endured hardship.” (Tahir Shah, In Search of King Solomon's Mines)

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

6 comments:

  1. hi sir may FB forums come , u r blog is unique in that its like a fiction novel reading experience, only in narayana we get to see cases like this impossible curves , collaterals ets and new way of LV prepration in TGA WITH IVS.

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  2. Dr Mukesh Kumar Singh, consultant pediatric cardiologist at Baroda, Gujarat and an extremely avid academician had the following comments to make:

    "DILV with DORV" is a 'common' entity, as far as such entities go. It is also consistent with accepted embryologic models. Purists could label it "DILV with double-outlet from an outlet chamber of RV morphology". Or liberals may safely assume (by logic of exclusion) that the smaller chamber in the ventricular mass is RV, given that the dominant chamber satisfies the definition of LV.

    ALCAPA: In an old post, you have highlighted a very important point. Echo can be treacherously misleading in the diagnosis of ALCAPA. Diagnostic uncertainty is further intensified by the presence of pulmonary hypertension after there is severe LV dysfunction and MR with raised LVEDP. LRTI, commonly the precipitating intercurrent illness, may also raise PVR and PA pressures. Somehow, this reappearance of PAH in the young infant (after the transition from neonatal high PVR physiology) does not sustain left coronary perfusion; although it certainly masks many echo signs. Literature describes and experience corroborates that ECG changes are always present even before echocardiographic findings. However the "pathologic" Q waves may be limited to a single lead (we saw a classic example of this - in lead aVL in one case). RCA: Ao root ratio may prove useful. All in all, it may be difficult to exclude ALCAPA sometimes even after a thorough evaluation.

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  3. Hi sir nice to read ur articles always new things to ponder keep it up. regarding TGA Intact IVS have u come across mild subpulmonic obstruction preparing LV?

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    Replies
    1. Thanks for the comment, Arun.
      In 2009, we presented a paper on TGA intact IVS with LVOT obstruction (sparing pulmonary valve) preparing LV for late ASO in PCSI (Dr Ritesh Sukharamwala). At that point, we had 17 cases. Can send you the soft copy if you are interested.

      Keep commenting!

      Kiran

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  4. Hi Kiran, happy to see the blog reborn. Keep up the good work in stimulating the brain with matters of the heart, Sudeep

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