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First Ever case of Shone Complex in India

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Dr Fazal Nabi & Dr Suresh Joshi [Jaslok Hospital] with 4 yr old Wilson from Nigeria operated for rare heart condition SHONE COMPLEX at Jaslok Hospital_1

Four year old Master Wilson Falohun [from Nigeria] came to Jaslok Hospital for the treatment of his heart condition. Wilson suffered shortness of breath and could not play like a normal child. He was also not putting on weight and was very small for his age. He was admitted under Dr.Fazal Nabi (Consultant Pediatrician) who has managed the patient initially and it was found that he had a hole in his heart which can be closed by open heart surgery. The patient was subsequently referred to Department of Cardio Thoracic Surgery. However, after investigating, Dr Suresh Joshi, Director, Dept. Of Cardiovascular & Thoracic Surgery, Jaslok Hospital found that he had a very complex and extremely rare heart condition called SHONE COMPLEX. There are less than one hundred such cases reported till date in world literature.

This rare operation has taken place ahead of the World Heart Day [29 September 2013] which aims to highlight prevention of cardiovascular disease with a focus on women and children.

In this condition, there is an obstructing membrane above the left sided Mitral valve which itself is abnormal and resembles a parachute and hence called the Parachute Mitral Valve. Also, there is severe narrowing and in this particular case, total blockage of the main blood vessel supplying to the entire lower part of body [Coarctation of Aorta]. In addition, there was a large PDA – an abnormal blood vessel supplying impure blood to the lower part of the body. Further, both great vessels supplying blood to the whole body and lungs were seen arising from the right side of the heart “ called Double Outlet Right Ventricle, and thus there was a very large defect in partition of the lower two pumping chambers.

Dr Suresh Joshi, Director, Dept. Of Cardiovascular & Thoracic Surgery, Jaslok Hospital explained, The operation was performed in one stage by two incisions. Initially, a closed heart surgery was done by making a cut on the left side of his chest to correct the narrowing of the blood vessel supplying to the lower body, along with closure of PDA. After completing this, the patient was turned flat and open heart surgery was performed by making the next cut on the front of his chest, to correct the remaining defects within the heart.

The open heart operation consisted of correction of the Mitral Valve defect, removal of obstructing membrane and closure of the large hole in the lower partition with a patch of patients own pericardium (a bag of tissue in which the heart is normally encased in our body). The hole was closed in such a way to direct the left sided blood vessel to the left pumping chamber and the right sided blood vessel to the right pumping chamber, thus restoring the normal circulation.

Wilson responded very well to the operation and was kept in the ICU for three days. About a week after his operation, he is now ready to go home to Nigeria, and in all probabilities he will not need any further treatment for his heart condition.

Birth Defects of Heart are always managed by Pediatric Heart Surgeons especially the complex defects like this patient, although in absence of specialist surgeons, adult cardiac surgeon may mange simpler form of birth defects; It takes a team of specialist besides pediatric heart surgeon to manage these types of case, which includes pediatric anesthetist, pediatric cardiac intensivist and pediatric cardiologists.

Pediatric cardiac surgery is a highly specialized super specialty and Jaslok Hospital has always strived towards taking up complex cases. It maintains the legacy of offering quality and competent medical care in such cases, says Dr.Tarang Gianchandani (Acting CEO, Jaslok Hospital).

Jaslok Hospital & Medical Research Centre is one of the few multi-specialty hospitals in the country which has a special department for Pediatric Cardiac Surgery, headed by Dr Suresh Joshi [Cardiovascular &Thoracic Surgeon].

Four year old Wilson, whose father works for the Central Bank of Nigeria, was sent to Jaslok Hospital for treatment thanks to the timely intervention of Dr Mrs. F.A.O Bello, Director of Medical Services at the bank, through their trusted medical facilitators in Mumbai, USHTA MEDICARE.

SHONES COMPLEX is an extremely rare congenital heart disease described by Shone in 1963. It consists mainly of a set of four left-sided cardiac defects, namely: supravalvular mitral membrane (SVMM), parachute mitral valve, subaortic stenosis (membranous or muscular) and coarctation of the aorta. Of these four defects, supravalvular mitral membrane (SVMM) is the first to occur, and triggers the development of the other three defects. It is an obstruction caused to the inflow and outflow of the left ventricle. So, the prognosis depends on the degree of obstruction that is being caused to the flow of blood. The diagnosis of Shones Complex requires an ultrasound of the heart (echocardiogram) and a cardiac catheterization procedure. The prognosis of Shones syndrome is poor, but in recent times, due to the advances in cardiothoracic surgery, the survival rate has increased.

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