In a first-of-its-kind surgery in India, Cardiothoracic Vascular Surgeons at Vadapalani, headed by Director and Senior Consultant - Institute of Cardiac & Advanced Aortic Diseases, SIMS, Hospitals, Vadapalani, performed an extremely challenging Aortic Root and Arch Replacement Surgery on a 66 year male from Andhra Pradesh, presenting with Situs Inversus Totalis, a rare medical condition, in which the patient's internal organs were reversed or mirrored from their normal positions.
Patient History and Line of Treatment
The 66-year-old gentleman presented with severe chest pain and cough to a local hospital in Andhra Pradesh. Surprisingly, the local doctor looking at the Chest Xray diagnosed him with Situs Inversus Totalis - An extremely rare clinical condition from birth, in which all the right-sided organs are present on the left side and the left-sided organs of the body are present on the right side. In short it is a case of mirror anatomy. To complicate further, the patient also had an acute Type A Aortic Dissection requiring a high risk emergency surgical procedure, in addition to severe long standing infection in both the lungs (Chronic Bronchiectasis).
Owing to the rarity of his medical condition and the need for a challenging and complicated surgery, (as surgeons they were not well-versed in operating such a patient and the hospital not well equipped to meet such rare condition), the local doctors promptly referred him to SIMS Hospitals, Vadapalani, Chennai.
Elaborating on the case and the rare condition of the patient, Dr VV. Bashi Director and Senior Consultant - Institute of Cardiac & Advanced Aortic Diseases, said, "Situs Inversus is a very rare medical condition, reported in 0.01 per centof the population or about 1 person in 10,000, where it seldom causes any symptoms and is so rare that the person may not even know they have it. They will continue to live a standard healthy life, without any complications related to the medical condition until they visit a doctor for a different reason.
In this case, the patient was diagnosed with acute Type A Aortic Dissection due to his severe chest pain and cough. He had bilateral bronchiectasis. The CT Aortogram revealed his anomalous anatomy, where his major organs, including heart, liver, gallbladder and spleen were on the exact opposite side of where they should have been. They all were functioning normally, which perhaps was the reason the patient didn't know he was a 1 in 10,000 human for seven decades. After performing all the necessary medical tests, the patient was planned for surgery. His aortic dissection was extending from the aortic valve to the abdominal part of the aorta. His aortic valve was having severe leakage as well.
Hence, he was planned for complete replacement of intrathoracic aorta - called as Bentall's procedure with aortic arch replacement. During the course of the 5 1/2 hours surgery, we did face several challenges owing to the rarity of the case and that no such operation was done earlier on a patient with Situs Inversus Totalis. As surgeons we had to orient ourselves to operate from the opposite side (mirror image) of the normal process to change the patient's aortic valve, ascending aorta and aortic arch, (the one which supplies blood to the brain, head, neck and arms) - so as to prevent the risk of decreased blood flow to the organs or the rupture of the aorta, which could potentially be life threatening to the patient. Today, after a month we are glad that the patient has recovered well post the surgery and is doing fine going about his daily routine.
To our knowledge, this is the first Major aortic operation done in a patient with bilateral bronchiectasis with Situs Inversus Totalis and Dextrocardia (Kartagener syndrome) in India."
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( With inputs from ANI )
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