Srinagar: A very complex cardiac surgery of a 6 year old child suffering from rare Genetic disorder (William – Beuren Syndrome) was performed at SSH Jammu.
An official of the GMC Jammu told KNO, that the six-years-old child presented with breathlessness on exertion and palpitations and was not gaining weight for his age.
He had impaired hearing, large ears, chronic right ear discharge, Eye problems, small chin (jaw), poor dentition, small widely spaced teeth with dental caries, umbilical hernia, undescended left testis & mild hydronephrosis, he said.
Notably incidence of William Syndrome is 1 in 10000 births and is associated with Cardiac (heart) defects and Aortic abnormalities.
The Patient had large hole in the wall between Lower chambers of the heart (Ventricular septal defect) causing large amount of blood with pressure getting shunted from left ventricle to right ventricle causing enlargement of Right Ventricle and Pulmonary artery, he said, adding that pressure on right side of heart and lungs was increased while he had decreased muscle tone and many biochemical abnormalities.
With so many associated morbidities, the surgery and peri-and postoperative care posed a great challenge both for Cardiac anaesthesiologists and Cardiac Surgeons, he said.
With informed consent, the patient was taken up for heart Surgery and the defect (hole) in the heart was repaired on cardiopulmonary bypass using a patch made up of material polytetrafluoroethylene (PTFE), he said.
The postoperative period was eventful in the patient, as he could not be weaned off from ventilator, early because of inherent weakness of muscles and known and unknown biochemical abnormalities and he was finally taken off ventilator on third postoperative day when his muscle power regained to maintain spontaneous respiration, he said.
He shall need further surgeries in future for Undescended left testis and right ear surgery, besides surgery for Umbilical hernia.
“Such good outcome in these high risk cases, is only possible because of dedicated team effort of Cardiac Anaesthesia in the postoperative Period in Intensive Care Unit,” Chief Surgeon at SSH Dr Shyam Singh said.
The Cardiac Anaesthesia team comprised of Professor & Head of department and Principal GMC Doda Dr Puja Vimesh, Dr Rasmeet Kour, Dr Vikas supported by senior Residents Dr Supriya, Dr Medha, Senior technologist Shivkaran Singh, Vikas Sharma, Saleem, Arif, Karan, Sonal, Abhiranjan and dedicated ICU Sisters Asma, Nayeem, Syeda, Zarat, Sunita, Shivalika, Vandana, Nighat, Sonakshi, Ekta, Shivani, Pooja, Urmila, Rajinder, Neetu and Staff, lead by Incharge Tejinder Kour. Surgical team comprising Dr Shyam Singh, Dr I. A.Mir, Dr Arvind Kohli, Dr Mohit, Dr Vivek and residents Dr Himani and Dr Shomaila Jan.
Senior Perfusionist Charanjit Singh and Roshan Lal maintained the Cardiopulmonary bypass parameters on the Heart Lung machine when the patient was being operated upon.
Senior Scrub Sisters Sapna Manhas, Arjumand ,Rigzen Angmo , assisted by Susheel, Sunil, Deepak, lead by incharge sister Vishali and senior technologist Girdhari Singh helped to carry out this high risk surgery.
Principal Professor Dr Shashi Sudan lauded the efforts of the Department of Cardiothoracic Surgery and Department of Cardiothoracic Anaesthesiology to make such surgeries possible, and routine in SSH Jammu under Ayushman Bharat Sehat Scheme absolutely free of cost. She further said that Open heart Surgeries are routinely done at SSH & the Patients are being benefited as all the Surgeries are done free for the Patients under Ayushman Bharat Sehat Scheme.
She further said very shortly the CTVS Deptt. shall also start CABG (Byepass Surgeries) and DNB vascular & CTVS streams.
