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

Optimal dose of adenosine effective for supraventricular tachycardia in children

To determine the optimal adenosine dose effective in supraventricular tachycardia [SVT] and underlying conditions affecting the effective dose in children. Experimental study. Department of Cardiology, The Children's Hospital and Institute of Child Health, Lahore, from July 2008 to June 2011. All children presenting with SVT were administered adenosine in rapid boluses according to PALS guidelines using incremental doses of 100, 200 and 300 micro g/kg. The response was recorded on 12 lead ECG. Preexcitation was documented and echocardiography performed on all children after attaining sinus rhythm. Mann Whitney test and Kruskal-Wallis test were used as a test of significance to determine any difference in effective adenosine dose between normal heart and various underlying conditions, taking p < 0.05 as significant. Eighty five patients were treated for 110 episodes of SVT with adenosine. M:F ratio was 2.2:1. Their age ranged from 6 days to 14 years with mean age of 27.9 months. Adenosine was effective in reverting 97 episodes of SVT to sinus rhythm [88.2%]. A dose of upto 100 micro g/kg was only effective in 36.4% episodes of SVT. Two hundred micro g/kg was effective in 44.3% of those not responding to 100 micro g/kg dose [n = 31/70, cumulative 64.5%]. A dose of 300 micro g/kg was effective in further 25 patients not responding to lower doses [n = 25/38, 65.8%; cumulative 88.2%]. Mean effective dose of adenosine was 185.3 +/- 81.0 micro g/kg with median effective dose of 200 micro g/kg. Significantly higher dose of adenosine was required in children with underlying pre-excitation, n = 18/97 [220.8 +/- 67.6 micro g/kg vs. 177.2 +/- 81.9 micro g/kg, p = 0.039]. Adenosine is an effective medicine in treating SVT in children. A higher dose of 200 micro g/kg may be used as first bolus particularly in children with pre-excitation

Socioeconomic status and impact of treatment on families of children with congenital heart disease

To assess the socioeconomic status, treatment being offered and the impact of congenital heart disease treatment on families. Observational study. The Children's Hospital / Institute of Child Health, Lahore, from 1st March to 31[st] August 2010. All patients undergoing a cardiac surgical or angiographic intervention were enrolled. Socioeconomic status was assessed by Kuppuswamy socioeconomic status scale with income group modification. The impact was measured by the source of financing, effect on family financing source and schooling and health of siblings. Of 211 patients undergoing treatment in the study period, surgery was the definitive treatment in 164 [77.7%] and angiographic intervention in 47 [22.3%] patients. Male to female ratio was 1.5:1. The mean age of the patient was 39.1 +/- 3.2 months [range 01 day to 15 years]. Majority of families belonged to middle [66.4%, n=140] and lower [27%, n=57] socioeconomic class. The mean cost of medicines and disposables was PKR 78378.2 +/- 8845.9 [US$ 933.1 +/- 105.3] in open heart surgery, PKR 12581 +/- 7010.8 [US$ 149.8 +/- 83.5] in closed heart surgery and PKR 69091 +/- 60906 in angiographic interventions. In 63.1% patients, families contributed towards these costs either completely [12.3%] or partly [50.8%] with significant contribution from the hospital. Adverse effect on families ranged from leave without pay to losing jobs or business [46%], and selling their assets [11.3%]. It also affected schooling and health of siblings [22.7% and 26.1% respectively]. Majority of children with congenital heart disease belonged to middle and lower socioeconomic status in this study. Main definitive treatment was surgery. The cost of health care facilities posed a marked socioeconomic burden on those families

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

Anatomical variations of pulmonary artery and associated cardiac defects in tetralogy of Fallot

To determine pulmonary artery variations and other associated cardiac defects in patients with Tetralogy of Fallot [TOF]. Cross-sectional, descriptive study. The Children's Hospital and the Institute of Child Health, Lahore, from April 2006 to October 2007. All patients with TOF, who underwent cardiac catheterization during this period, were included. Standard cine-angiograms were recorded and pulmonary artery sizing was done using z-scoring. A total of 216 patients with TOF were catheterized. Pulmonary Artery [PA] abnormalities were present in 84 [38.9%] patients. The commonest abnormality was isolated Left Pulmonary Artery [LPA] stenosis [n=27, 32.14%] followed by isolated hypoplasia of Main Pulmonary Artery [MPA] [n=18, 21.43%] and supra-valvular stenosis in [n=11, 13.1%] patients. LPA was absent in one patient, while 2 patients had both absent right and left PA with segmental branch pulmonary arteries originating directly from MPA. Associated cardiac lesions included right aortic arch in 34 [15%], additional muscular VSD vary in 13 [5.5%], Patent Ductus Arteriosus [PDA] in 11 [6%] and Major Aortopulmonary Collateral Arteries [MAPCA] in 2 [1.9%] patients. Significant coronary artery abnormality was present in 10 [4.6%] children. Pulmonary artery abnormalities were present in 38.9% of patients with TOF. Isolated LPA origin stenosis and MPA hypoplasia were the most common abnormalities. Significant associated cardiac lesions were present in one-third of the patients and included PDA, additional muscular VSD, coronary artery abnormalities and MAPCA