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Posts Tagged ‘rabinovitch’

Great things happen in India nowadays  .Economy is growing    at  9% , the growth of  automobile industry is  fastest in the world. But in  scientific  research and development  it   has  been traditionally lagging behind . Things are set to change. The medical science , (If you want to call it as industry it is fine !)  especially  the imaging science is making rapid strides. The proliferation of  private and corporate hospitals and institutes   has helped us practice the latest .

Pulmonary atresia with VSD is a rare congenital disease where  there is partial or  total (chaotic  )pulmonary arterial blood supply .

When the pulmonary  artery becomes atretic , what will the lung do ?

It has to get perfused somehow ! It tries to snatch the  blood from the aorta  in whatever  possible manner . Depending on the severity of pulmonary atresia  , there  can be  a total anomalous pulmonary  arterial supply (Type 4 ) .Here , few twigs directly originate  from Aorta, few from branches of  Aorta  and sometimes bilateral  PDA etc .These are collectively called major arotopulmonary collateral  (MAPCAS) .In fact differentiating a PDA from a MAPCA can be extremely difficult .(It has only academic purpose though !)

Hither to ,  visualising  the  MAPCAS was a  huge  task . Aortogram with selective cannulation of MAPCA  was  neccessary.Now,  with the advent of  MDCT we can get some stunning  images  of these collaterals non invasively.

Why is visualizing and delineating MAPCA anatomy important ?

This will facilitate the surgeon to plan  the  unification of pulmonary  arterial flow    and reenginnering the pulmonary circulation  (with or with  out  conduits)

Here is a rare  publication originating from India  in  American journal of radiology  . An  exclusive   article  with  CT scan  images of the  defect .

Amrita  Institute , from  the southern   Indian  state of  Kerala (  also known as God’s  own country )  is doing a phenomenal  cardiology  work  especially  in pediatric cardiology.

Three cheers to the team which published this  master piece . With the courtesy of   AJR the link to the article is placed here.

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                                  Even as cardiology community is preoccupied with systemic hypertension & CAD  ,  pulmonary arterial hypertension(PAH) is a much neglected , still  an important clinical cardiac problem encountered . The irony is self evident , there are half a dozen methods to grade systemic hypertension not even a single stadardised grading available for pulmonary arterial hypertension. The WHO  working group defined pulmonary hypertension  few decades ago and was not clinically graded .The only grading available is based on  the pulmonary vascular biopsy changes (Heath Edwards) 

                                   Currently PAH management has gone through revolutionary changes. There is an urgent  need for grading  this entity .This will facilitate to  diagnose , manage and assess the efficacy of the currently available treatment.

                                Developing countries like ours have a great number of PAH due to rampant rheumatic heart disease.  A simple study was done in  100 patients with PAH .Bulk of the study population had RHD .Few had primary pulmonary  hypertension .Systolic , diastolic, and mean pressure was assessed by doppler echocardiographic analysis of tricuspid regurgitation (TR) and pulmonary regurgitaion(PR) jets. TR jet provided the systolic PA pressure , PR jet provided mean as well as diastolic PA pressure .TR jet was available in all patients. PR jet was available only in 60 patients .Hence the diastolic andmean PA pressure data has been extrapolated in some  and  was plotted in a scatter diagram. Five equal quintiles were divided. Patients in first  and 2nd quintiles were graded 1   and third  and 4th  quintile were  graded 2 ,  5 th  was graded 3 respectively. From this cut off points for  various grades of PAH were identified .The top 3% of patients  with highest PAP were graded as grade 4 and all of them had supra systemic PAH. 

The following grading is suggested for PAH* 

 *This is a preliminary  attempt to grade PAH. This could be applicable mainly in rheumatic heart disese and primary pulmonary hypertension .Further refining of methodology is  required.PAH grading may be little different in congenital left to right shunts.

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