Excelsior Correspondent
JAMMU, Apr 27: A very complex cardiac surgery on a 6-year-old child suffering from a rare genetic disorder (William – Beuren Syndrome) was performed at Super Speciality Hospital (SSH) Jammu.
The 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 and mild hydronephrosis.
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. Pressure on right side of heart and Lungs was increased. Moreover, 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. With informed consent, the Patient was taken up for heart surgery. The defect (hole) in the heart was repaired on cardiopulmonary bypass using a patch made up of material polytetrafluroethylene (PTFE).
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. He was finally taken off ventilator on third postoperative day when his muscle power regained to maintain spontaneous respiration.
The Cardiac Anaesthesia team was comprised of Professor and Head of Department Dr Puja Vimesh, Dr Rasmeet Kour and Dr Vikas while the surgical team had Dr Shyam Singh, Dr IA Mir, Dr Arvind Kohli, Dr Mohit and Dr Vivek.
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