Hemophilic Patient For Emergency Spinal Decompression

Hemophilia is mostly an inherited genetic disorder, caused by mutations in the clotting factor gene. With the available treatment options, life expectancy of a hemophilic patient is usually of that of the general population. Hence, it is not uncommon that they present for surgical procedures. However, hemophilic patients for the major surgical procedure are always a real challenge for the perioperative physician. We have recently encountered one such patient who was hospitalized with acute paraplegia due to a mass lesion of spine and successfully managed with the recovery of motor power. His pre-operative Factor VIII level was 0. Desmopressin nasal spray has a limited role in severe hemophilic. Our main concern was effective replacement therapy and maintenance of desired Factor VIII levels not only during surgery but also in the immediate post-operative period

Criss-cross heart: Transthoracic echocardiographic features

Objective To study the echocardiographic features of criss-cross heart (CCH), a congenital cardiac anomaly characterized by crossed ventricular inflow streams, in Indian patients. Methods In this retrospective observational study, all pediatric echocardiograms performed in a single tertiary care institution in South India over a three-year period were scrutinized for a diagnosis of CCH. Demographic, clinical and echocardiographic data were collected from patients’ medical records and echocardiographic database. Crossed ventricular inflow streams was identified when there was inability to visualize both atrio-ventricular valves in a single imaging plane in cardiac four chamber view. Results CCH was diagnosed in five patients from 10,500 pediatric echocardiographic studies. The age at diagnosis ranged from one month to 8 years. Cyanosis was present in all but one of the five cases. Crossed ventricular inflow streams was present by definition in all cases, whereas superior-inferior ventricular relationship was present in only three cases. All cases were associated with ventricular septal defects. Atrio-ventricular discordance was seen in three cases and concordance in two. Ventriculo-arterial discordance was seen in three cases, concordance in one and double outlet right ventricle in one. Three cases had pulmonary stenosis and the other two had pulmonary arterial hypertension. Straddling of AV valve was observed in four cases and hypoplastic aortic arch in one case. Conclusion CCH is an extremely rare congenital cardiac anomaly. Superior-inferior ventricular relationship often co-exists with CCH, but is not necessarily present in all cases. CCH requires early diagnosis because of its common association with diverse cardiac anomalies.

Tumour necrosis factor (TNF)-α-308 gene polymorphism in Indian patients with Takayasu’s arteritis - A pilot study.

Background & objectives: Tumour necrosis factor-alpha (TNF-α)- 308 promoter gene polymorphism has been shown to be associated with several autoimmune disorders and infections such as tuberculosis. There is no study on TNF-α gene polymorphism in Takayasu’s arteritis (TA) till date. We aimed to study this polymorphism in TA, a granulomatous vasculitis, probably triggered by Mycobacterium tuberculosis. Methods: TNF-α - 308 gene polymorphism was studied in 34 patients with TA and 39 healthy controls recruited from Christian Medical College, India. PCR was done followed by enzyme digestion. G and A polymorphisms were analysed. Occurrence of alleles in the disease group was compared with controls as well as with historical controls. Results: GG allele was most frequent in TA and in controls. GA allele was detected in four controls but only in one patient who was the oldest in the study group. AA polymorphism was detected in one control but not in TA. When compared with controls from other populations, it was found that our allelic frequency was similar to that in Japan as well as from USA with mixed population. However, predominantly Caucasian population studied from Netherlands, Germany and England, where TA is rare, had a higher frequency of A allele as compared to our controls. Interpretation & conclusions: Our preliminary results indicated that G allele at TNF-α - 308 was more common in TA patients and controls similar to that in other Indian as well as Japanese population. Compared to the western population, A allele was relatively less common in our study subjects.

Incessant atrial flutter after device closure of atrial septal defect: successful radio frequency ablation.

A four-month-old baby presented with failure to thrive and congestive cardiac failure precipitated by a lower respiratory tract infection. He was found to have a large ostium secundum atrial septal defect measuring 18 mm. This was successfully closed percutaneously by a device (Blockaid). A month after the device deployment the child developed typical atrial flutter. Despite rate control drugs the ventricular rate remained l40/min over the next several months. In view of the incessant atrial flutter with fast ventricular response, the child underwent radiofrequency ablation at the age of 2 years. An isthmus block was created which successfully terminated the tachycardia.

Prevalence of coronary artery disease in patients with rheumatic heart disease in the current era.

BACKGROUND: This study was undertaken to determine the prevalence of coronary artery disease in patients with rheumatic heart disease undergoing valve surgery. METHODS AND RESULTS: Consecutive patients with rheumatic heart disease (n=376) who were above the age of 40 years, and scheduled for valve surgery underwent diagnostic coronary angiogram to delineate coronary arteries. The patients were divided into three groups based on valve involvement (mitral valve, aortic valve, and combined aortic and mitral valve). Significant coronary artery disease was considered to be present if one or more coronaries showed 50% or more luminal stenosis. There were 287 (76.3%) males and 89 (23.7%) females. The mean age of the study population was 51.2+/-8.2 years. Eighty-nine (23.8%) patients had typical chest pain, 116 (30.6%) patients had atypical chest pain and 171 (45.5%) patients had no chest pain. Hypertension was noted in 88 (23.4%) patients, 65 (17.3%) patients had diabetes, 98 (26.1%) patients were smoker, and 66 (17.6%) patients had dyslipidemia, and 15 (4.0%) patients gave past history of myocardial infarction. Of the total 376 patients, 46 (12.2%) patients were found to have significant coronary artery disease. In patients with mitral vale disease the prevalence was 13.5% (13/96), while it was 15.3% (19/124) in patients with aortic valve disease and 9% (14/156) in those with combined mitral and aortic valve disease. CONCLUSIONS: Our results suggest that the overall prevalence of coronary artery disease in a group of patients with rheumatic heart disease undergoing valve surgery in the current era is 12.2%. This prevalence is much lower than the figures reported earlier in the Western literature.

Multivessel cutting balloon angioplasty in a patient with type III nonspecific aortoarteritis.

A 23-year-old female patient with type III nonspecific aortoarteritis (Takayasu's arteritis) presented with multiple obstructive lesions and severe congestive heart failure. Large, cutting balloons 5-8 mm in diameter were used to dilate lesions in the abdominal aorta, both renal arteries, right common carotid artery, proximal left subclavian artery, and ostium of the left vertebral artery. Wide luminal expansion without residual stenosis, substantial dissection or need for adjunctive stenting was achieved at all six angioplasty sites. The use of cutting balloons appears suitable for treating obstructive lesions in aortoarteritis.