Double orifice mitral valve: A rare cause of isolated severe congenital mitral regurgitation.

Double orifice mitral valve (DOMV) is a rare congenital anomaly of the mitral valve apparatus consisting of an accessory bridge of fibrous tissue, which partially or completely divides the mitral valve into two orifices. It usually occurs as an associated lesion. Encountering a DOMV as an isolated finding meriting intervention is indeed rare. We, here, report a case of "complete bridging type DOMV" occurring as an isolated entity and presenting as severe congenital MR requiring surgery.

Congenital giant right coronary artery.

Giant coronary artery aneurysms are exceptionally uncommon with an incidence of 0.02%. The natural history and prognosis of giant coronary artery aneurysm are still not well known.

An intriguing case of post-operative respiratory failure from an occult diaphragmatic hernia- be aware of the masquerader.

Though respiratory complications after cardiac surgery for congenital heart disease are common, and malformations of the diaphragm can be expected in these patients, the presence of an occult diaphragmatic defect unrecognisible preoperatively and complicating the post operative course is very rare and need a high index of suspicion for diagnosis in the setting of post operative respiratory failure. We present here a case of post operative respiratory failure from a delayed presenting diaphragmatic hernia in a 2-month-old boy who underwent corrective surgery for Taussig bing anomaly and hypoplastic aortic arch. Surgical repair of the diaphragmatic defect and reduction of the bowel loops to the abdomen resulted in rapid weaning from ventilation and recovery with subsequent discharge from hospital.

Cardiac syncope from occult transposition masquerading as convulsive epilepsy.

A four-year-old boy on Valproate therapy for a presumed diagnosis of epilepsy presented with a recurrence of tonic spasms. A history of precipitation of these episodes with exercise prompted a search for an underlying cardiac disorder. The child was ultimately diagnosed to have corrected transposition of great arteries with 2:1 atrioventricular block. This case illustrates that cardiac syncope due to underlying congenital heart disease can masquerade as epilepsy in children.

Brainstem auditory evoked responses in term small for gestational age newborn infants born to undernourished mothers.

Our aim was to assess the effect of intrauterine growth retardation on neurosensory development by evaluating brainstem auditory evoked responses (BAER) in term small for gestational age (SGA) newborn infants born to undernourished mothers. This prospective clinical study included 25 singleton healthy SGA newborn infants born between 38 and 41 weeks to undernourished mothers (weight <45kg, height <145cm, haemoglobin <8g/dl, and serum albumin <2.5g/dl). An equal number of age- and sex-matched appropriate for gestational age newborn infants born to healthy mothers served as controls. Mothers with other risk factors and newborns with complications during delivery or immediate newborn period were excluded. BAER was recorded within first 3 days of life. Interpeak latency (IPL), absolute peak latency (APL) and amplitudes of various waveforms were determined and compared between the groups. No statistically significant differences were observed for the mean interpeak and absolute latencies between term SGA and AGA infants (p>0.05). The absolute peak latency (wave V) and central conduction time (I-V interval) were borderline prolonged in the study group compared with controls (p=0.051 and 0.088 respectively). Using multiple regression analysis, maternal haemoglobin was identified to be the only parameter having a negative correlation with both IPL (waves I-V) (F[1,46]=4.12, p=0.048) and APL (wave V) (F[1,46]=5.80, p=0.02). Maternal undernourishment may have a minor effect on intrauterine development of the auditory brainstem. Maternal haemoglobin is the only factor significantly associated with these changes.