Balloon valvuloplasty in dysplastic pulmonary valve stenosis: immediate and intermediate outcomes

To determine the immediate and intermediate outcome in dysplastic and doming pulmonary valve stenosis in children and to determine various factors associated with unsuccessful outcome. An interventional study. The Children's Hospital, Lahore, Pakistan, from June 2006 to December 2012. All patients presenting with severe pulmonary valve stenosis were enrolled in the study. Balloon valvuloplasty was performed on all patients. Successful outcome [residual gradient < 36 mmHg] was compared with matched doming pulmonary valve stenosis control group valvuloplasty. Difference in various quantitative variables was calculated using independent t-test and Mann Whitney U test. Categorical variables were compared using Chi square and Kruskal-Wallis test. Multivariate analysis was performed to determine various factors associated with outcome. Kaplan- Meier survival table was used to determine freedom from re-intervention proportions. One hundred and fifty two patients [Dysplastic group A, n=73; Doming group B, n=79] with median age of 24 months [range 3 - 192 months] and M:F; 2:1 were included in the study. Mean gradient decreased from 96 +/- 33 mmHg to 29 +/- 20 mmHg. Group A had significantly higher number of patients with unsuccessful outcome [9.6%, p=0.02]. Preprocedure gradient > 75 mmHg was the most significant factor associated with unsuccessful outcome [p < 0.001]. Median follow-up duration was 3 years [range 1 - 6 years]. Freedom from re-intervention proportion at 1, 3 and 6 years was 91.3%, 86.7% and 78.9% respectively in group A compared to 100%, 96.5% and 96.5% respectively in group B. Immediate postprocedure gradient > 60 mmHg was the only significant factor associated with re-intervention in group A [p=0.001]. The results from balloon valvuloplasty in dysplastic pulmonary valve were suboptimal when compared to doming valves. However, it provides a high freedom from re-intervention rate in intermediate follow-up. Intervention at moderate severity can result in better outcome

Congenital heart defects associated with atrial heterotaxy

To determine the frequency of various congenital cardiac defects associated with atrial heterotaxy and the difference between spectrum of cardiac defects in right atrial isomerism [RAI] and left atrial isomerism [LAI] in these patients. Cross-sectional observational study. Department of Cardiology, The Children's Hospital and Institute of Child Health, Lahore, from July 2006 to June 2011. All children presenting to the hospital with suspected congenital heart defect were included in the study. Echocardiography based sequential segmental analysis was used, taking atrial symmetry, interrupted inferior vena cava [IVC] and juxtaposed aorta to IVC relation into account for identifying atrial heterotaxy. Various segmental defects were noted for RAI and LAI. Children had atrial heterotaxy [M:F; 1.7:1] with 61.6% [n=82] having LAI. Most common lesions associated with RAI included complete atrioventricular [AV] septal defect [n=48, 94.1%], single AV valve [n=45, 88.2%] and pulmonary outflow tract obstruction [n=41, 80.4%]. LAI was associated with ventricular septal defect [n=68, 82.9%], atrial septal defect [n=63, 76.8%, mostly secundum variety], and miscellaneous left sided obstructive lesions. Atrial heterotaxy encompasses a wide spectrum of congenital cardiac defects. The frequency of various defects associated with RAI or LAI in local South Asian population of Lahore, Pakistan is similar to those as reported in the Western literature

Pattern of ventricular Septal defects and associated complications

To determine the frequency of various types of Ventricular Septal Defect [VSD] in local population and their complications. Case series. The Pediatric Cardiac Unit of the Children Hospital and Institute of Child Health, Lahore, from January 2006 to December 2007. All patients aged between one day to 18 years presenting to the unit with isolated VSD during the study period were evaluated using 2-D, continuous wave Doppler and colour Doppler techniques on transthoracic echocardiography. Where necessary, transesophageal echocardiography and/or angiography data was used to further clarify the anatomy. Of the 854 patients with isolated VSD, 710 were of perimembranous type [83.1%], 100 were muscular type [11.7%], 25 were doubly committed subarterial [DCSA] type [2.9%] and 19 patients were having inlet VSD [2.2%]. The mean age was 2.1 +/- 3 years. Females were 332 [38.8%] and males were 522 [61.1%]. Aortic Valve Prolapse [AVP] was seen in 77 patients [10.8%]. The mean age for this subgroup was 5.8 +/- 4 years with 54 [70.1%] males and 23 [29.9%] females. Of those, 36 [47.3%] had various degree of aortic regurgitation. Right ventricular outflow tract obstruction of variable severity was found in 4 patients. Two of these patients had echocardiographic evidence of subacute bacterial endocarditis. Perimembranous [PM] VSD is the commonest type of ventricular septal defect presenting to a tertiary care hospital. The incidence of AVP and AR is high [10.8%] and was found almost entirely in perimembranous outlet VSDs rather than DCSA. This observation has special implication in Pakistan where pool of unoperated VSDs is large