Mixed variety of total anomalous pulmonary venous connection: diagnosis by 2D echocardiography and Doppler colour flow imaging.

Of the many types of total anomalous pulmonary venous connection, mixed type is the least common. Its accurate non-invasive diagnosis by echocardiography poses a diagnostic challenge. We report our experience of echocardiography in 21 infants with mixed type of total anomalous pulmonary venous connection aged 25 days to one and half years. Multiple windows were used to identify individual pulmonary veins and various sites of drainage. Cardiac catheterisation and angiography were performed for 17 cases. In 11 of 21 cases, the left upper pulmonary vein was seen draining into vertical vein and the left lower and right-sided pulmonary veins were draining into the coronary sinus. Cardiac and supracardiac combinations of other types were seen in eight more cases. Both drainage sites were supracardiac in one case and supracardiac and infracardiac in another. On comparing echocardiographic findings with those obtained at cardiac catheterisation and/or surgery (carried out in 18 cases), there were three instances of error. In two cases (echocardiography performed without the use of colour flow imaging) the second site of drainage could not be defined. These patients were catheterised as all four pulmonary veins were not delineated by echo. The third error occurred in a case where although two sites of drainage were picked up by echo but at surgery, the right lower pulmonary vein was noted to have a double connection, both to coronary sinus and to vertical vein. We conclude that mixed type of total anomalous pulmonary venous connection can be accurately diagnosed by echocardiography and Doppler colour flow imaging. The diagnostic errors are rare and would not alter the surgical management.

Prostaglandin E1 in infants with congenital heart disease: Indian experience.

BACKGROUND: E-type prostaglandins (PGE1) can effectively maintain the patency of the ductus arteriosus in neonates. Its use, therefore and be life saving in infants born with ductus dependent congenital heart disease. Although PGE1 is available for over two decade in western world, it has been introduced in India only since April, 1995. OBJECTIVE: To assess the efficacy of PGE1 at our center. SETTING: Hospital based. METHOD: 65 infants with ductus dependent congenital heart disease were included. Age at time of starting PGE1 infusion ranged from 18 hours to 39 days. Forty two of these were more than a week of age, 19 were more than 14 days, and two were above one month. PGE1 was started in an initial dose of 0.05 microgram/kg/min, decreased to 0.005-0.01 microgram/kg/min for maintenance. The indications for use of PGE1 were to increase pulmonary blood flow in 33 cases with pulmonary atresia, tricuspid atresia or critical pulmonic stenosis (Group I); to increase systemic blood flow in 15 cases with coarctation of aorta, hypoplastic left heart and interruption of aortic arch (Group II); to improve mixing in 13 cases of transposition of great arteries (Group III) and for improving the left ventricular volumes by keeping the duct open in 4 cases of transposition of great arteries with intact ventricular septum (Group IV). The efficacy of the drug was assessed by a rise on PaO2 and SaO2% determined for Group I & III, and by appearance of lower limbs pulses in Group II. Left ventricular volumes were serially measured by echocardiography in Group IV cases. RESULTS: The drug was successful in 62 of the 65 cases. There were two failures. One was a 39 days old baby with Ebstein's anomaly of tricuspid valve and pulmonary atresia and other was an eight days old baby with coarctation of aorta and renal failure. In addition, PGE1 could not be continued in another baby due to development of a linear skin rash locally. Side effects included apnea in 5 (9%) of 56 spontaneously breathing patients. Necrotizing enterocolitis, hyperpyrexia and jitteriness was sent in one case each. Six patients died. Two were related to PGE1, one due to failure, another due to its side effects. Definitive procedure were performed in 51 cases electively. PGE1 was used upto 13 days with sustained benefit. CONCLUSIONS: PGE1 is an effective drug for keeping the ductus open in infants with ductus dependent congenital heart disease. It can be used for neonates beyond the first week of life with efficacy. Apnea is a major side effect and close monitoring is essential.

Aortic valve balloon dilatation in infants with critical aortic stenosis.

Twenty-six infants aged 10 days-11 months (mean 3.5 +/- 2.4 months), with critical aortic stenosis underwent aortic valve balloon dilatation in last six years. In 19 (73%) infants, aortic valve balloon dilatation had to be performed as an emergency procedure. The systolic gradients decreased from 71.7 +/- 11.8 mm Hg to 21.0 +/- 8.1 mm Hg. There was no procedural death. Severe aortic regurgitation developed in two (7.7%) which was medically managed. Four (15.4%) infants had femoral artery thrombosis following aortic valve balloon dilatation and all responded to intravenous streptokinase. Congestive heart failure resolved in all (100%). Two infants developed endocarditis during follow-up after aortic valve balloon dilation and one of them died, another patient died of severe pulmonary artery hypertension. In the surviving 24 infants, left ventricular function improved markedly. On follow-up of 18 +/- 5 months, restenosis developed in two (7.7%) patients. We conclude that aortic valve balloon dilatation is safe and effective treatment for infants with critical aortic stenosis and severe left ventricular dysfunction.

Balloon dilatation of simple aortic coarctation in neonates and infants.

Twenty-one infants with isolated native coarctation of aorta (mean age 4.73 +/- 2.85 months, range 15 days-12 months) underwent balloon dilatation in the last seven years at our institute. Seven of them were less than three months and 14 were older than three months. The procedure was successful in relieving the coarctation (gradient < 20 mm Hg) in five of seven (71%) infants up to three months and 12 of 14 (86%) above three months. Peak systolic gradients decreased from 70.8 +/- 20.1 to 14.5 +/- 11 mm Hg in the younger infants and from 47.7 +/- 11.7 to 11.4 +/- 9.9 mm Hg in infants above three months. Post-dilatation angiogram showed the coarctation segment to be equal to the isthmus in both the groups, 0.96 +/- 0.14 and 0.96 +/- 0.12 respectively. One three months old infant died immediately after a successful dilatation possibly due to coronary embolism, and another neonate died a few days later of unrelated pre-existing septicemia. Left ventricular function improved in all the infants with depressed preprocedure ejection fraction. Restenosis defined as peak instantaneous echo-Doppler gradient above 20 mm Hg with a significant narrowing on two-dimensional echocardiography occurred in all the five infants up to three months over a period of 1 to 12 months. Follow-up information over a period of 4 to 24 months was available in 11 of 14 older infants of which three had restenosis. Gradients progressively increased in all the four infants (2 of either group) with an initial partially successful result. A small isthmus relative to the descending thoracic aorta at diaphragm is an important predictor of restenosis/inadequate result. This discrepancy was seen much more in smaller infants, post-dilatation coarctation index being 0.68 +/- 0.11 vs 0.81 +/- 0.16 (p = 0.07). Four of the infants underwent a repeat successful dilatation. Balloon dilatation is safe and effective in infants above three months of age. In infants up to three months the procedure is feasible and leads to improvement in left ventricular function, but restenosis occurs rapidly in all of them.

Prostaglandin E1: first stage palliation in neonates with congenital cardiac defects.

E-type prostaglandins (PGE1) can effectively maintain the patency of the ductus arteriosus in neonates. Its use, therefore can be life saving in infants born with ductus dependent congenital heart disease. Although PGE1 is available for over two decades in western world, it has been introduced in India only since April, 1995. Various cardiac defects where PGE1 is useful include (a) lesions with ductus dependent pulmonary blood flow e.g. pulmonary atresia with or without ventricular septal defect, critical valvular pulmonic stenosis etc, (b) lesions with ductus dependent systemic blood flow e.g. critical aortic stenosis, coarctation of aorta, interruption of aortic arch etc, and (c) admixture lesions like transposition of great arteries. The drug is given as a continuous intravenous infusion. The initial dose is 0.05 to 0.4 ug/kg/min, infusion rate must be decreased to 0.01 ug/kg/min as soon as the desired effect is achieved as incidence of side effects is more at higher doses. Serious side effects include apnoea, hypotension, hyperthermia, seizures etc. We have used this drug in 43 infants ranging in age from one to forty five days. Beneficial response was seen in 41 of 43 infants and the major side effect was apnoea (seen in 5 of 32 spontaneously breathing infants). Unfortunately the high cost of the drug prohibits its wide spread and long term use. PGE1 is a life saving drug for infants born with ductus dependent congenital cardiac malformations. It helps in stabilizing these patients prior to further surgical palliation or correction.

Right ventricle to pulmonary artery connections.

From March 1988 through June 1995, 80 patients underwent repair for right ventricle (RV) to pulmonary artery (PA) discontinuity. Of these, 30 patients received homograft conduits, while the remaining 50 underwent repair without the use of a homograft. Handmade pericardial valved vascutek conduits (n = 18), non-valved vascutek tubes (n = 13) and non-conduit procedures like the rev operation (n = 12) and extended pericardial gusset (n = 5) were mainly used. The indications for repair for RV-PA discontinuity included ventricular septal defect with pulmonary atresia (VSD PA) (n = 36), truncus arteriosus (n = 18), ventricular septal defect with pulmonary and major aorto-pulmonary collaterals (VSD PA MAPCA) with staged repair (n = 5), congenitally corrected transposition of the great arteries with ventricular septal defect and pulmonic stenosis (CCTGA VSD PS) (n = 5) and double outlet left ventricle with ventricular septal defect and pulmonic stenosis (DOLV VSD PS) (n = 5). The choice of the procedure was governed by various factors, including type of cardiac defect, pulmonary artery pressure and morphology, previous operation, socio-economic constraints and availability of appropriate sized homografts. Non-circuit operations may prevent future reoperations in these patients.

Conjunctival impression cytology in dry eye states.

Impression cytology technique was used to study the cytological changes of in 30 patients with various diseases (Xerophthalmia, Steven-Johnson's syndrome, trachoma, alkali burns, kerato conjunctivitis sicca) resulting in dry eye syndrome. The main features of impression cytology were squamous metaplasia of epithelial cells and altered goblet cell density. The comparison between conjunctival biopsy findings and impression cytology confirm that impression cytology provides the same information as that of biopsy. Hence, the authors advocate that this non-invasive simple technique can replace conjunctival biopsy in confirmation of diagnosis in dry eye syndrome.

Results of penetrating keratoplasty with cataract extraction and intraocular lens implantation in Fuchs' dystrophy.

Combined procedure was performed in 25 eyes and triple procedure in 32 eyes of Fuchs' dystrophy. Pre-operative visual acuity was 20/100 or less in 63.2% of eyes. The graft was clear in 76% of combined procedure and in 97% of triple procedure eyes. Post-operative visual acuity of 20/40 or better was obtained in 57.9% of eyes. The common cause of graft failure was allograft reaction. The other causes attributable to visual failure were opacification of posterior capsule, secondary glaucoma, macular degeneration and pre-existing glaucomatous cupping. Intraocular lens insertion at the time of surgery helps in rapid visual rehabilitation.