Surgical experience with congenital heart disease in Down's syndrome.

Children with Down's syndrome and congenital heart defects have multiple problems. The role of cardiac surgery in the management of these patients was investigated by reviewing the clinical data, hospital course and follow-up of 21 patients (9 males and 12 females, age range 1 month to 14 years) with Down's syndrome and congenital heart defects operated in our institute. Twelve (57%) of these were infants and nine (43%), older children. Five were in congestive cardiac failure, four were hypothyroid. The heart lesions ranked in incidence as follows: atrioventricular septal defect 7 (33.3%), tetralogy of Fallot 3 (14.3%), tetralogy of Fallot & atrioventricular septal defect both 2 (9.5%), double outlet right ventricle with pulmonary stenosis 1 (4.8%), patent ductus arteriosus 2 (9.5%), patent ductus arteriosus plus coarctation 1 (4.8%), ventricular septal defect 2 (9.5%), atrial septal defect plus ventricular septal defect 1 (4.8%), atrial septal defect plus patent ductus arteriosus plus right pulmonary artery stenosis 1 (4.8%) and transposition of great arteries with multiple ventricular septal defect 1 (4.8%). Four (19%) patients had palliative procedures while the rest (81%) underwent primary repair. All survived the operation. The post-operative period was complicated in 6 (28.5%), with respiratory infections in 3, pulmonary hypertensive crisis in 2 and complete heart block in 1. The early mortality was 0, while there were 2 (9.5%) late deaths. The number of hospitalisations was markedly reduced according to the parents. Follow-up showed near normal pulmonary artery pressure in 50 percent children with large shunts and a good developmental spurt was seen in 60 percent. From a purely surgical viewpoint, the prognosis for children with Down's syndrome and congenital heart disease is good.

Atrial entrapment of flow directed pulmonary artery catheter-a technique for non-surgical removal.

A 43 year old patient who underwent mitral valve replacement had pulmonary artery catheter inserted before induction of anaesthesia. The catheter could no be removed postoperatively by routine manoeuvres in the intensive care unit. Fluoroscopy in the cardiac catheterization revealed a straight course of the catheter indicating the probability of its inclusion in the left atrial suture line. The pulmonary artery catheter was successfully removed percutaneously in the cardiac catheterization laborartory. The procedure is described.

Bidirectional Glenn shunt: a step in the right direction.

Bidirectional Glenn (BDG) shunt was carried out in 14 patients (age range 1.5-22 years; mean 9.3 years) for a variety of congenital cyanotic heart diseases with decreased pulmonary blood flow. Postoperative cardiac catheterisation was carried out in 10 patients including successful balloon angioplasty of the shunt and LPA in one patient. There was a significant improvement in oxygen saturation and a drop in haematocrit level at follow-up. Doppler echocardiography studies in 13 patients revealed functioning shunts in all cases with low velocity continuous flow. Thus BDG is a useful palliative procedure and could be undertaken as the first stage of total cavopulmonary connection in high-risk Fontan groups where biventricular repair is not feasible.

HLA and Disease Susceptibility in Tamil Nadu, S. India.

Result on HLA polymorphism in the populations of Tamil Nadu, South India are analysed for HLA association with various diseases like pulmonary tuberculosis, leprosy, psoriasis, rheumatoid arthritis, iridocyclitis and Eale's disearse. These studies revealed: i) association of psoriasis with HLA B17 and DR7 is highly significant in Vellala related group and this is attributed to founder effect-hitch hiking of a disease producing gene linked to these alleles, as the population migrated, ii) different castes differ from one another in their HLA allelic and haplotype polymorphism, iii) HLA DR2 predispose for more severe from of pulmonary tuberculosis which transcends ethnic barrier, the susceptibility presumably being due to a generalized MHC dependent immunogenetic and pathological mechanism, iv) HLA B27 and DR4 are associated with iridocyclitis, rheumatoid arthritis and low back pain; the susceptibility may be a HLA dependent molecular mimicry process, v) HLA alleles DR2 and DR4 predispose for severe forms of the respective diseases, though not for initial susceptibility.

Prevalence of sickle cells in Irula, Kurumba, Paniya & Mullukurumba tribes of Nilgiris (Tamil Nadu, India).

A total of 1377 tribals, comprising Irulas (536), Paniyas (196), Kurumbas (87), Mullukrurumbas (156) and Soligas (402), living in the Nilgiris, Tamil Nadu, India were studied for sickle cell trait between 1981-85. Patients attending various tribal clinics at Arayure, Kozhikarai, Kothagiri and Biligiri Rengan hills for various ailments were screened at random by solubility test and by acetate paper electrophoresis, if required. HbAS carrier frequency was 30-37.8 per cent in all the tribals studied except Kurumbas (19.5%). The frequency of carriers were more (37.8%) on the western part of Nilgiris (Nedungode, Kappala and adjoining regions) than the eastern part (30%). Further, the prevalence of carriers was higher (47-49%) in the 10-19 yr age group amongst Paniyas and Mullukurumbas living in the western part of Nilgiris. An episodic, epidemic of malaria so rampant during the early part of this century in the western parts of Nilgiris might have eliminated many children with HbAA and hence the higher frequency of HbAS in this particular age group.

Blade and balloon atrial septostomy for creation of an adequate interatrial communication.

During a 7 year period from 1984 to 1991, 100 infants underwent either balloon atrial septostomy (BAS) (n = 92) or blade septostomy (BLS) (n = 8). The indication was complete transposition of the great arteries in all the patients. The mean age in the BAS group was 1.8 +/- 1.5 months while that in the infants requiring BLS due to a thick septum was 3.03 +/- 2.29 months. The degree of improvement in arterial oxygen saturation in both groups was satisfactory -27.16 +/- 14.06% in the BAS group vs 23.5 +/- 12.18% in the BLS group. There were no procedure related deaths in the BLS group, through only monoplane fluoroscopy was used in 6/8 patients. Three patients however died following balloon septostomy. We conclude that BLS is a safe and effective alternative to surgical septostomy when performed with due care. Two dimensional echocardiography during BAS enhances the speed and safety of the procedure and helps to identify patients who may require BLS due to a thick interatrial septum.

Primary pulmonary hypertension: natural history and prognostic factors.

A retrospective cohort study was carried out in 61 patients (30 males, 31 females, age 24.6 +/- 11.8 years) with primary pulmonary hypertension diagnosed by strict clinical and hemodynamic criteria, to obtain an understanding of the natural history and prognostic markers. While 15 patients were alive, 46 patients (76%) had expired during the follow up period. Two, five and ten years survivals were 48%, 32% and 12% respectively. Median survival duration from time of diagnosis was 22 months. The survivors had significantly higher age of onset, cardiac index and significantly lower right atrial mean pressure, right ventricular end diastolic pressure, cardiothoracic ratio from chest rontgenogram and calculated pulmonary vascular resistance as compared to non survivors. While pulmonary artery systolic pressure was not significantly different, pulmonary artery diastolic and pulmonary artery mean pressures were significantly lower in survivors than in non-survivors. Lower New York Heart Association class, right atrial mean pressure < or = 7 mm Hg, right ventricular end diastolic pressure < or = 10 mmHg, cardiac index > 2.5 L/min/m2, pulmonary arterial oxygen saturation > 60%, were associated with significantly longer survival. The degree of pulmonary arterial hypertension had an indirect prognostic effect through the above parameters. Vasodilator therapy did not significantly alter the outcome of patients with primary pulmonary hypertension.

Thrombolytic therapy for prosthetic cardiac valve thrombosis.

During 1992-93 12 patients (5 males, 7 females) with thrombosed prosthetic cardiac valves were treated with streptokinase on 13 occasions (one patient with prosthetic tricuspid valve had two thrombotic episodes). Their age ranged from 14 to 52 years (median 39). Two valves were in aortic position, six in mitral and four in tricuspid position. Eight were Bjork-Shiley prosthesis, three were Medtronic Hall valves and one was a St. Jude Valve. Timing of prosthetic valve thrombosis ranged from 3 months to 12 years after valve replacement surgery. Duration of symptoms due to valve thrombosis ranged from 1-4 months with tricuspid valve thrombosis and 1-14 days with left sided valve thrombosis. Five were in functional class II and four each were in functional class III and class IV. All patients were evaluated by echo Doppler and cine fluoroscopy. Loading dose of streptokinase was 2.5 lakh units in 4 patients and 1 lakh units in 9 patients. Maintenance infusion was at 1000 units/Kg/hour in 11 patients and 1 lakh units/hour in 2 patients. Duration of streptokinase infusion ranged from 3 hr to 38 hr. Thrombolytic therapy was successful (clinical, echo Doppler and fluoroscopy) in 12 out of 13 cases (92%). It was unsuccessful in a patient with valve at tricuspid position in whom infusion had to be stopped after 24 hour due to bleeding gums. One patient developed intracerebral bleed and expired. In conclusion streptokinase therapy is useful for prosthetic cardiac valve thrombosis.

Enhanced lipid peroxidation in patients during coronary artery bypass grafting.

Lipid peroxidation products were measured at various time intervals in 20 patients with coronary artery disease, who underwent coronary artery bypass graft (CABG) surgery. Post-operative blood lipid peroxides were found to be significantly higher (p < 0.001) than the preoperative value. Lipid peroxides raised to a peak value of 46.42 +/- 12.86 n mol/g Alb at 5 min of reperfusion when compared to the basal value and afterwards the level declined to 41.02 +/- 7.09 at 2 hrs and remained in that level even at 24 hrs of reperfusion. This increase implies an enhancement in free radical mediated oxidation of membrane lipids during bypass surgery and thus provides evidence for free radical generation during myocardial reperfusion.

Frequency of HLA antibodies in south India.

Sera from 4088 pregnant women (649 antenatal bleeding and 3439 post-partum bleeding) living in Madurai, were collected and screened for anti-HLA. Α, Β and DR antibodies. 696 of them were screened for anti-HLA DR antibodies. Ten per cent (65/649) of antenatal sera and 13·4% of post-partum sera (463/3439) were positive for HLA A and Β antibodies: nonetheless the percentage of monospecific sera were almost the same in both. Screening for HLA DR antibodies were carried out using platelet absorption in test tray technique: seventy three of 696 (10·5%) were positive. The incidence of anti-HLA A, B antibodies correlates to the allelic frequencies in the population. Thus in India, collection and screening post-partum haemorrhage is the simplest and cost effective method of acquiring polyclonal sera for routine laboratory and diagnostic use.

Distribution of cholesterol in HDL and its subfractions in patients with coronary atherosclerotic heart disease.

The distribution of HDL-C and its subclasses HDL3-C and HDL2-C and other serum lipids was studied in patients with coronary atherosclerotic heart disease grouped as young males (group 1) and older males (group 2) along with age matched controls. All the patients had significantly higher levels of serum cholesterol, triglycerides, LDL-C and VLDL-C and lower levels of HDL-C. The analysis of HDL - subclasses clearly demonstrated that the low levels of HDL-C was due to the significant decrease of cholesterol in both HDL3 (group 1: 32%; group 2: 30%) and HDL2 subclasses (group 1: 55%; group 2: 48%) compared to the respective control values. Further it has also been observed that this low level of HDL-C is a characteristic feature of patients irrespective of whether the levels of serum cholesterol and triglycerides are high or normal. Although both the HDL subclasses were decreased, the percentage of reduction of cholesterol was greater in the HDL2 than in HDL3 subclass. In addition, the levels of cholesterol either in HDL or any of its subclasses, HDL3 and HDL2 did not show any change in relation to the extent of coronary disease which was assessed by coronary angiography. This study confirms the inverse relation of HDL-C with coronary atherosclerosis and also indicates that, of all the lipid parameters examined, only HDL-C, particularly its subclass HDL2-C, shows independent inverse relation to the incidence of coronary atherosclerotic artery disease in men.

Two dimensional and Doppler echocardiographic evaluation of double chambered right ventricle.

Two-dimensional and Doppler echocardiographic findings in 20 patients with double chambered right ventricle are described. All patients had the diagnosis established by cardiac catheterisation and confirmed at operation. Echocardiographic evaluation was done prior to surgical correction. Anomalous muscle bands in right ventricular cavity were detected in 16 patients. Doppler flow velocities in the right ventricular cavity suggested infundibular obstruction to blood flow at a low level in all 17 patients studied by Doppler echocardiography. Ventricular septal defects (11 patients), pulmonary stenosis (3 patients), and aortic regurgitation (3 patients) were detected accurately before operation by echocardiographic examination. Failure to detect the anomalous muscles in right ventricular cavity may occur in adult patients with poor anterior resolution and in those with severe right ventricular outflow obstruction and myocardial hypertrophy. Two dimensional echocardiography with Doppler flow analysis is useful in the evaluation and differential diagnosis of right ventricular outflow obstructions prior to invasive studies and surgical intervention.