Eponyms in Cardiology starting with N
By : Dr. Subrahmanyam Karuturi
Naxos disease is a recessively inherited condition with arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) and a cutaneous phenotype, characterised by peculiar woolly hair and palmoplantar keratoderma. The disease was first described in families originating from the Greek island of Naxos. Moreover, affected families have been identified in other Aegean islands, Turkey, Israel and Saudi Arabia. A syndrome with the same cutaneous phenotype and predominantly left ventricular involvement has been described in families from India and Ecuador (Carvajal syndrome). Woolly hair appears from birth, palmoplantar keratoderma develop during the first year of life and cardiomyopathy is clinically manifested by adolescence with 100% penetrance. Patients present with syncope, sustained ventricular tachycardia or sudden death. Symptoms of right heart failure appear during the end stages of the disease. In the Carvajal variant the cardiomyopathy is clinically manifested during childhood leading more frequently to heart failure. Mutations in the genes encoding the desmosomal proteins plakoglobin and desmoplakin have been identified as the cause of Naxos disease. Defects in the linking sites of these proteins can interrupt the contiguous chain of cell adhesion, particularly under conditions of increased mechanical stress or stretch, leading to cell death, progressive loss of myocardium and fibro-fatty replacement. Implantation of an automatic cardioverter defibrillator is indicated for prevention of sudden cardiac death. Antiarrhythmic drugs are used for preventing recurrences of episodes of sustained ventricular tachycardia and classical pharmacological treatment for congestive heart failure, while heart transplantation is considered at the end stages.
In contrast with Rastelli procedure, the Nikaidoh procedure creates a more anatomically correcct left ventircular outflow tract. Also know as aortic translocation and biventricular outflow tract reconstruction. Nikaidoh procedure is used for patients born with a combination of transposition of the great arteries, ventircular septal defect and pulmonary stenosis.
The Norwood procedure is a series of three open-heart surgeries that gradually improve certain life-threatening forms of congenital heart disease. It is used most often to treat hypoplastic left heart syndrome, but variations of the procedure may also be used to treat other congenital heart diseases in which one or both of the lower chambers of the heart (ventricles) are defective. Each of the three surgeries is done at a different age, beginning from infancy and spanning through the toddler years. The first two surgeries (Stage I and Stage II) are used to temporarily relieve blood flow problems to and from the lungs. The third surgery (Stage III) is used to further improve circulation. The Norwood procedure cannot cure the underlying heart defects.
The Norwood procedure re-routes the blood flow around some of the defective areas of the heart by creating new pathways for blood circulation to and from the lungs. Despite the complexity of the procedure, many children go on to live a relatively normal lifestyle after completing all three stages.
The most common heart defects that are treated by the Norwood procedure are: Hypoplastic left heart syndrome (HLHS). Some types of tricuspid atresia. Various other faulty connections among the upper and lowers chambers of the heart, resulting in the heart’s inability to maintain good blood circulation throughout the body.
More Cardiac Eponyms