Atrial septal defect is an abnormality present in a person from birth where there is a hole in the partition between the upper chambers of the heart called the atria. As a rule many times if it is small it can close on its own by 2yrs of age beyond that[a1] if it is of simple nature then one comes to know of it only in second decade. If the type of this defect is atypical which is called Sinus Venosus type of defect then symptoms happen earlier and needs early intervention also. Here most of times one vein carrying pure blood from lungs which was to drain into left upper chamber is opening into right upper chamber this increasing the flow to lungs. Or some time all veins from right lung are opening into the superior Vena Cava (Vein draining impure blood from upper parts of body to right upper chamber) along with large gap in the partition between the two upper chamber. In such a situation the flow to the lung increases markedly and permanent damaging changes start setting in and a stage comes when resistance of lung vasculature increases so much that blood flow direction reverses. Normally it is left to right but then it becomes right to left and patient start becoming blue also. This is a stage where surgery is not possible and patient become in-operable. Otherwise if heart surgery for this carried out at the right age and time is suitable and patient becomes absolutely normal for rest of his /her life.
Recently a 21yrs female who had given birth to a child 4 months back presented to us at Alchemist Hospital, Panchkula with severe breathlessness and palpitation. On examining we found her to be having a hole in the heart in both the upper chamber and investigating her with echo-cardiography and going through her previous record we found that she had atypical sinus venous type of atrial septal defect which manifested after child birth but along with her pressure in the lung vasculature had become very high almost equal to the body’s blood pressure. In view of this outside other peripheral hospitals had denied her surgery in view of very high risk including the risk to life. Infact she was sent Delhi for opinion.
When she presented to us we also felt the same way but then we decided to further investigate looking at her young age. The only chance for correction or treatment through surgery was now and otherwise it could be fatal. We admitted her and did a cath study on her. The lung pressures had peaked to 110mmHg which was equal or slightly more than her own blood pressure and shunt across the defect got reduced to 1.3:1 (normal criteria for surgery is shunt more than 1.5:1 or 2.1). The pulmonary vascular resistance came out to be 12 wood units, close to the terminal limits, where one becomes in- operable. Only positive finding in the study was that she was still maintaining almost normal oxygen levels in left side of upper chamber i.e left atrium about 97% that was a sign that she was not de-saturating but other parameters pointed towards non-operability.
We decided to plan her treatment and give her a chance. We started her on certain newer drugs to lower her lung pressure before surgery, talked to the family of high risk involved and total picture and need to prepare her for about a week before surgery by drugs including lungs pressure lowering drugs, to flush out extra fluid from body and to give rest to right heart and also some thing to improve the contractions of right heart.
Looking at the literature again she was in that rare group where surgery was not possible. After nine days of preparation we decided to do a repeat echocardiography and she did responded to medication but marginally and also there was no bluish discoloration involved, so we felt that changes were not irreversible. On 17.06.2011 she was operated for open heart surgery and her hole was closed with a patch made out of the outer layer of heart called pericardium, diverting all the right lung veins which were connecting to lower part of superior Vena Cava to the left. We did one more innovation in creating a flap- valve type of patch in her case. The idea of this is that incase after surgery the right heart pressure increases the flap valve allows the right chamber to dicompresss by opening up. Heart muscle was protected well during surgery with various newer Techniques. She responded well to treatment and surgery and her lung pressure came down to 50% of arterial pressure in the immediate post- op period. They further were controlled with the new drugs which we had started earlier in the pre- op period. Over all she responded very well and lung pressure almost came to normal range.
She was discharged on 8th day with detailed explanation about the medications and precaution and on her follow up visit on 1st June it was very heartening to see her healthy and progressing well. Well thought of strategy and extra effort in treatment goes a long way in saving these high risk patients along with a good and skilled infrastructure and highly experienced team of doctors. 6 Senior doctors were involved in her care on day to day basis and it was very satisfying for all of us to save this young life. She was operated by a team headed by Dr Varinder Sarwal, Head- Dept. of CTVS, Alchemist hospital, Panchkula, Dr Arath Nahak, Dr Ajay Sinha, Dr Deepak Oberoi, Dr N Srivastava and Dr Dheer
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