Dr Kiran welcomes everyone again to the blog of Pediatric cardiology department, Narayana Hrudayalaya Hospital, Bangalore.

I read a short-story published about 3 decades back in one of the Indian magazines. I found it worth sharing: There was an honest man in an office. He meant his work, did it with full sincerity, never gave a chance for anyone to complain. In a chance of event, the management brought in a person with lesser skills, lesser knowledge and higher ego above this honest man. This new person, who was not even one tenth capable, was drawing double the salary of the honest man. Although nothing else changed, the new entry broke the honest man from inside. He started comparing his skills with the new person. He started counting all the contributions he had made to the office over years. He started comparing the strengths and weakness of both and started calculating how much more salary he should command. The sole objective of him coming to the office became discussing this with every single employee of the office including the watchman, lift-man, tea-supplying boy, office-peon and so on. The management was so deft that everytime he logically tried to explain his point, they got their ego hurt and made extremely frivolous remarks on this honest man! He lost peace of mind and thereby the zeal for work. He just became another useless person in the system, gossiping about the impotency of management in rewarding the skills and capability. He was no more what he was so far. This “Avada Kedavra” effect is aptly titled “Murder of righteous”

Such systematic murders are very rampant. Nepotism, caste, influence, glamour and everything other than honesty and principles seem to work! It may be impossible to retain a sane frame of mind to most. To add on, there are people in the system, whose sole objective is to unearth such issues and kill the peace of mind of others! Individual integrity and high degree of self motivation is not easy to achieve. One needs to face the mirror and keep telling oneself about the perpetual need for retaining their honesty and integrity. These testing times have become the rule in today’s corporates. Almost everyone goes through this experience. What is the opinion of readers? How do you retain the sanity in the light of these adverse realities?

I came across an interesting article in online issue of JTCS. This was an accepted manuscript I found online in the first week of March 2014. It is titled “A simple surgical technique for closure of apical muscular VSD” and authored by Dr Amit Mishra et al from U. N. Mehta Institute of Cardiology and Research Center, Ahmedabad, India. Dr Mishra was very kind in allowing the review of this article for our blog.

The authors have presented their innovative and simple surgical technique using custom made low profile polytetrafluoroethylene (PTFE) single disc device for closing multiple apical muscular and isolated apical muscular ventricular septal defects in 17 children. Two children with significant co-morbidities could not be saved out of the cohort of these17 children. The authors have detailed the steps for the preparation of these custom-made devices in their centre.

After echo guided sizing and location of VSDs, the authors have used right atriotomy, opening of IAS, approaching the LV side of VSDs via mitral valve. They have placed the device across the IVS and have sutured it to the septal muscles adjacent to the device. This, they have reported, takes about 15-20 minutes time.

This appears commendable. For, apical VSDs are always delicate issues! Heavy trabeculations on the RV side limits the visibility. Lack of space to work with in the cardiac apex is another limiting factor. Despite early success of left ventriculotomy for dealing with apical VSDs, the risk of aneurysm formation looms large on long-term follow up. The sandwich technique has the risk of residual VSDs.

The authors claim that their technique has the advantage over hybrid techniques in overcoming the limitations and complications of the latter. They also claim that the low profile and plasticity of their custom made device blends well with ventricular geometry in contrast to the commercial devices. Above all, the cost of this custom-made device is estimated by the authors to be about INR 6000 to 7000/- per piece, which is less than one tenth of the commercially available devices!

Any resource-limited country needs innovative approaches to cut the cost down without compromising on the safety. Once the scientific principles are solid and established, there should be way for innovation along the same principles. Now that we have walked a long way with the devices, it is time to innovate and cut downs the costs everywhere to enable utilizing resources to larger masses. Despite being limited by numbers, this paper by Indian colleagues appears way ahead and may be path-breaking. Time is the best umpire.

With that, let us get back to the interesting learning scenarios:

PREPARING WITH REGURGITATION

Among the causes of LV preparation in TGA with intact IVS, the roles of LVOT obstruction or aortopulmonary shunts are well known. We had one infant of 6-months old with TGA and intact IVS. This baby had a congenital mitral valve cleft, resulting in significant regurgitation. The LV was adequately prepared! The child had a successful arterial switch with good outcome. This is one of the rare causes of LV preparation. Please share your experiences on similar rare causes of LV preparation in cases of TGA.

HIERARCHY OF PRIOROTIES

Cardiac transplant in third-world countries is yet to find its place. Barring few anecdotal reports and success, it is still exception than rule. Right from legal issues to emotional issues, there are obstacles. Despite the availability of expertise, the raw-material is of premium. As a result, consensus guidelines are not even attempted. In such cases, how feasible is the advice for cardiac transplant in patients with single ventricles? Very frequently, we see patients of end-stage single ventricles referred from peripheral centres with advice for cardiac transplant. Many a times the advice would be so strong that the pateint’s attendants refuse to leave our chambers till they get “Yes”! It is difficult to understand why such options are given at first hand? Hierarchically, this category of patients does not make it to the top of priorities when the numbers of available hearts are very minimal. Is there a plausible way to deal with such issues? Please let us know your experiences on this issue.

TIMING THE EVENTUAL

We often come across children with CCTGA with VSD associated with pulmonary stenosis. However, the overall shunts would be balanced and these children would be maintaining good saturation with reasonable effort tolerance with no great symptoms. They would eventually require Double switch surgery with atrial switch, LV to aorta tunnel with RV to PA homograft repair. Despite being the corrective surgery, it is massive! Chances of re-do are very high. Many surgeons believe in postponing the surgery to as late as possible. Although the scientific guidelines differ, this approach appears to be very practical in third-world where the cost of surgery and re-do is totally on the parents. Also, the risk of re-do and eventual morbidity should also be accounted. What is opinion of readership? How do you tackle this issue? Where else can we follow this approach? Please let us know.

BIAS OF BACKWARD PRESSURE

It is always maintained that the PAH resulting from pulmonary venous hypertension is reversible. That is the main reason why surgeons bravely march when left-heart events are the sole cause of PAH. Barring cases of pulmonary venous anomalies, this analogy is true. Can the same be extrapolated to events with combination of pulmonary venous hypertension with high Qp? I am referring to TAPVC. It is not uncommon to see an adult with TAPVC! Many surgeons maintain that all TAPVC are operable irrespective of age!! In TAPVC, there is excess Qp capable of causing pulmonary vascular obstructive disease along the lines of ASD. At the same time, there is also some element of pulmonary venous hypertension due to back pressure. How to decide operability in such scenarios? As we all clearly know, cath study is useless in such cases. Is there any way of determining the operability when the findings are not straight forward? Would plethoric chest radiograph or good pulmonary venous returns are enough markers? I would invote the opinions of readers on this issue.

TOT-A-TOTE 

Earlier, we had discussed a child with d-TGA with intact IVS with cor-triatriatum leading to preparation of LV even at the age of 8 months. In contrast, we came across 1-month-old child with dTGA, intact IVS with regressed LV due to TAPVC with small ASD! This child had features of low Qp with good systemic saturations! Our surgeons commented that “half the Senning procedure already done”! LV was not exposed to pressure or volume load, causing early regression. Although many maintain that such LV can be trained, the outcome should be guarded. Not many of this combination would have made it to OT table! It would be interesting if the readers share their experiences on this issue.

With that we conclude this post. Please pen your comments in the comments section. If you find any problem in posting comments, please feel free to mail it to my email id kiran.vs.dr@nhhospitals.org I shall post them on your behalf.

Regards

Kiran