Current Clinical Profile of Acute Rheumatic Fever and Recurrent Acute Rheumatic Fever in Pakistan.

Inclusion of echocardiography as diagnostic tool and polyarthralgia and monoarthritis as major criteria for high-risk populations in the Revised Jones Criteria 2015 is likely to surface substantial variability in clinical manifestations among various populations. This study aimed to compare clinical profile of patients presenting with first and recurrent episodes of acute rheumatic fever (ARF) using most recent criteria. 130 consecutive patients with ARF were included in the study from August 2019 to March 2022. World Heart Federation standardized echocardiographic criteria were used for cardiac evaluation. The socio-demographic variables, clinical details and relevant investigations were recorded. Median age was 13(6-26) years. Male to female ratio was 1.6:1. Majority was of low socioeconomic status (90%) and with > 5 family members in a house (83.8%). 27 patients (20.8%) were with ARF while 103 (79.2%) with recurrent ARF. Carditis was the most common presenting feature (n = 122, 93.8%), followed by polyarthralgia (n = 46, 35.4%), polyarthritis (n = 32, 24.6%), subcutaneous nodules (n = 10, 7.7%), monoarthritis (n = 10, 7.7%), and chorea (n = 5, 3.8%). Monoarthralgia was more common in ARF than recurrence (29.4% vs. 3.2%, p = 0.004). Carditis (97.1% vs. 81.5%, p = 0.01) and congestive cardiac failure (18.5% vs. 5.9%, p = 0.001) were more common in recurrent ARF than ARF. Diagnostic categorization of Jones criteria for different populations has highlighted important variability in clinical presentation of ARF. Monoarthralgia is common in first episode of ARF. Carditis is the most common feature in recurrent ARF. Polyarthralgia is seen with higher frequency that polyarthritis. Subcutaneous nodules seem to be more common in our population.

Device closure of residual aortopulmonary window using Konar-MF occluder device: an attractive new option.

We report a case of a residual aortopulmonary window where a new Konar-MF occluder device was used to close the defect. This device has a low profile and conforms to the anatomy of aortopulmonary window very nicely without unnecessary protrusion on either side. This report highlights the advantage of Konar-MF occluder device for closure of such defects.

Short to midterm follow-up of multi-system inflammatory syndrome in children with special reference to cardiac involvement.

OBJECTIVES: We aim to describe the early and upto 16 months follow-up of post-coronavirus disease (COVID), multi-system inflammatory syndrome in children (MIS-C), with special reference to cardiac involvement. STUDY DESIGN: This cohort non-interventional descriptive study included patients <18 years admitted between May, 2020 and April, 2021. Based on underlying similarities, children were classified as post-COVID MIS-C with overlapping Kawasaki Disease, MIS-C with no overlapping Kawasaki Disease, and MIS-C with shock. Post-discharge, patients were followed at 1, 3, 6, 12, and 16 months. RESULTS: Forty-one patients predominantly males (73%), at median age of 7 years (range 0.2-16 years) fulfilled the World Health Organisation criteria for MIS-C. Cardiac involvement was seen in 15 (36.5%); impaired left ventricle (LV) function in 5 (12.2%), coronary artery involvement in 10 (24.4%), pericardial effusion in 6 (14.6%) patients, and no arrhythmias. There were two hospital deaths (4.9%), both in MIS-C shock subgroup (2/10, 20%). At 1 month, there was persistent LV dysfunction in 2/5, coronary artery abnormalities in 7/10, and pericardial effusion resolved completely in all patients. By 6 months, LV function returned to normal in all but coronary abnormalities persisted in two patients. At last follow-up (median 9.8 months, interquartile range 2-16 months), in 36/38 (94.7%) patients, coronary artery dilatation was persistent in 2 (20%) patients. CONCLUSIONS: Children with MIS-C have a good early outcome, though MIS-C with shock can be life-threatening subgroup in a resource-constrained country setting. On midterm follow-up, there is normalisation of LV function in all and recovery of coronary abnormalities in 80% of patients.

Percutaneous closure of ventricular septal defect using LifeTechTM Konar-MF VSD Occluder: initial and short-term multi-institutional results.

BACKGROUND: Transcatheter ventricular septal defect closure remains a complex procedure with potential complications like complete heart block and aortic regurgitation. The ideal device design for such intervention is still evolving. AIM: To assess the safety, efficacy, and short-term outcome of ventricular septal defect closure using LifeTechTM multifunctional (KONAR-MFTM) VSD Occluder. PATIENTS AND METHODS: In a multicenre study, 44 patients with haemodynamically significant, restrictive ventricular septal defects underwent closure with the KONAR-MFTM device from April, 2019 to March, 2020. Clinical, echocardiographic, and angiographic data were collected and reviewed. Patients were followed up at 1, 3, 6, and 12 months. RESULTS: The median age and weight were 8 (1.7-36) years and 20 (11-79) kg. Of 44 patients, 8 (18%) had a high muscular and 36 (82%) had a perimembranous defect, of which 6 had mild prolapse of the right coronary cusp. The median ventricular septal defect size was 8.8 (3.9-13.4) mm. A retrograde approach was adopted in 39 (88.6%) patients. Nine patients (20.5%) had a small residual leak and there was a slight increase in aortic regurgitation in one patient. One device, which embolised to pulmonary artery was retrieved, and the defect was closed with a larger device. At a median follow-up of 13 (5-18) months, the residual leak persisted in 1 (2.3%) patient. Mild aortic regurgitation in one patient remained unchanged. There were no major complications. CONCLUSION: Percutaneous closure of ventricular septal defect using KONAR-MFTM device is safe and effective in short and midterm follow-up including selected patients with perimembranous defect and mild prolapse of the right coronary cusp.

Antimicrobial resistance modulation of MDR E. coli by antibiotic coated ZnO nanoparticles.

We evaluated three types of total six preparations against multidrug resistant E. coli i) three antibiotic coated ZnO nanoparticles (gentamicin coated nanoparticle-GNp; chloramphenicol coated nanoparticles-CNp; and both gentamicin & chloramphenicol coated nanoparticle-GCNp), ii) ZnO nanoparticle alone-Np, and iii) two antibiotics used in single (Gentamicin-G; and Chloramphenicol-C). A total of n = 200 sub-clinically positive mastitic milk samples of bovine origin were processed for isolation of MDR E. coli using microbiological and clinical laboratory & standard institute's protocols. ZnO Nps were prepared from zinc acetate dihydrate (Zn (CH3COO)2. 2H2O), polyethylene glycol (C2nH4n+2On+1), and urea (CH4N2O) by standard chemical protocol. Nps were characterized by XRD and STEM analyses while coating of antibiotics on Nps was confirmed by UV-Visible spectrophotometric analysis. Analysis of variance and student t-test were applied at 5% probability using SPSS version 22 statistical software for inferences on obtained data. There was significantly (p < 0.05) lowest minimum inhibitory concentrations (MICs) and highest zone of inhibitions (ZOIs) in case of GCNp (10.42 ± 4.51 µg/mL & 22.00 ± 1.00 mm) followed by GNp (20.79 ± 8.95 µg/mL & 20.00 ± 1.00 mm) and then CNp (25.96 ± 8.95 µg/mL & 12.33 ± 0.57 mm). Percentage increase in ZOI were expressed as 135.8, 78.43, and 312.76% by GCNp when compared with that of G, C, and Np, respectively. GNp and CNp coated preparations exhibited 114.36 and 275.73% increase in ZOI than to that of G and C, respectively. Similar trend was found in percentage reduction of MICs of preparations. Highest filamentation, indicator of bacterial damage, of E. coli was noted at MIC of GCNp followed by GNp and CNp. The study concluded antibiotic coated ZnO nanoparticles significant candidates modulating antibiotic resistance in MDR E. coli.

Malposition of left atrial disc and feasibility of recapturing Figulla-II Occlutech septal occluder.

We report a case of Figulla-II Occlutech septal occluder malposition with residual shunt at posteriosuperior margin of an atrial septal defect. Improvising its bioptome type delivery cable, same system was used to recapture the device and redeploy it successfully. This report highlights a potential malfunction of Figulla-II Occlutech disc and the advantage of its delivery system for retrieval of the device.

Intermediate- and long-term follow-up of device closure of patent arterial duct with severe pulmonary hypertension: factors predicting outcome.

BACKGROUND: In patients with large patent arterial ducts and severe pulmonary hypertension, the natural history of progression of pulmonary hypertension is very variable. Whether to close or not to close is often a difficult decision, as there are no established haemodynamic parameters predicting reversibility. OBJECTIVES: The objectives of this study were to evaluate the results of device closure of large patent arterial ducts with severe pulmonary hypertension after 2 years of age and to determine haemodynamic variables associated with its regression during long-term follow-up. METHODS: A total of 45 patients, with median age of 10 (2-27) years, with large patent arterial ducts and severe pulmonary hypertension, were considered. Haemodynamic variables were assessed in air, oxygen, and after occlusion. The follow-up was performed to assess regression of pulmonary hypertension. RESULTS: Device closure was successful in 43 (96%) patients. Pulmonary artery systolic and mean pressures decreased from 79 to 67 mmHg and from 59 to 50 mmHg, respectively (p25% (both in oxygen) (p=0.007). CONCLUSIONS: Device closure of large patent arterial ducts with severe pulmonary hypertension is safe and effective. Pulmonary vascular resistance index and systolic and mean pulmonary artery pressures in oxygen are the key prognostic variables predicting regression of pulmonary hypertension.

Yield of 48-hour Holter monitoring in children with unexplained palpitations and significance of associated symptoms.

OBJECTIVE: To determine the yield of 48-hour Holter monitoring in children with unexplained palpitations and the significance of associated symptoms. METHODS: This descriptive study was conducted at the Children's Hospital and Institute of Child Health, Lahore, Pakistan, from January 1 to December 31, 2015. All children above 5 years of age with history of intermittent palpitations and normal basic cardiovascular workup were enrolled. A 48-hour Holter study was performed using Motara Holter Monitoring System. Frequency of various symptoms and abnormal Holter findings were analysed. SPSS 21 was used for data analysis. RESULTS: Of the 107 patients, 69(64.5%) were males and 38(34.5%) females. The median age was 10 years (interquartile range: 5-18 years). Most common concomitant symptoms with palpitation included syncope/pre-syncope in 35(32.7%) patients, chest pain 22(20.5%), shortness of breath 21(19.6%) and colour change/pallor 11(10.3%). Holter recording was positive in 40(37%) patients. Frequent premature ventricular contractions 12(11.2%) and atrial ectopic beats 9(8.4%) were the most common findings. Holter findings were significantly more common in patients with history of shortness of breath and colour change/pallor during palpitations (p=0.002). CONCLUSIONS: Extended 24-hour Holter monitoring in children with palpitations was an inexpensive, non-invasive investigation with a reasonably high diagnostic yield in detecting arrhythmias.

CHANGING CLINICAL PROFILE OF ACUTE RHEUMATIC FEVER AND RHEUMATIC RECURRENCE.

BACKGROUND: Clinical profile of acute rheumatic fever and rheumatic recurrence seems to have changed in countries where rheumatic fever is still endemic. The objectives of this study were to compare clinical profile and outcome of patients suffering initial and recurrent episodes of acute rheumatic fever in children. METHODS: This prospective study was conducted in two tertiary care hospitals from January to June 2011. The diagnosis was based on the modified Jones criteria. Sixty children were included in the study, 15 having first episode of rheumatic fever and 45 with rheumatic recurrence. The severity of carditis was assessed by Clinical and echocardiography means: RESULTS: Carditis was the commonest presentation in both first (80%) and recurrent attacks (100%). Arthritis was seen in 60% of children with first episode and in 26.7% with recurrence. The frequency of subcutaneous nodules, invariably associated with carditis, was very high (33.3% in the first and 48.3% in recurrent episodes). Carditis was generally mild during first episode (53.3%) and severe with rheumatic recurrence (55.6%). There was no death in either group. One patient with severe mitral regurgitation and rheumatic recurrence underwent mitral valve repair for intractable heart failure. CONCLUSION: Clinical profile of rheumatic recurrence and acute rheumatic fever has changed. Rheumatic recurrence is associated with severe carditis. Carditis is more common than arthritis even in the first attack. Sub-cutaneous nodules are a frequent finding invariably associated with carditis.

Covered stents in the management of native coarctation of the aorta--intermediate and long-term follow-up.

OBJECTIVES: To evaluate the results of covered Cheatham-Platinum stent implantation in the management of native coarctation of the aorta and report intermediate and long-term follow-up. BACKGROUND: Covered stents are being used increasingly in severe and complex coarctation of the aorta mainly to reduce risk of aortic wall complications. There is, however, limited data on the intermediate and long-term outcome. PATIENTS AND METHODS: Fifty-six patients received 59 covered Cheatham-Platinum stents (January 2002-December 2011) at a single center-in 54 patients as primary treatment and in 2 as a rescue. Mean age was 22.25 ± 1.2 (11-56) years and mean weight 58.6 ± 2.1 (32-99) kg. Primary end points were reduction in systolic pressure gradient and an increase in coarctation segment diameter. Changes in antihypertensive medicines and complications were recorded on follow-up. RESULTS: Mean coarctation segment diameter increased from 4.69 ± 0.20 to 15.1 ± 3.2 mm (P < 0.0001). The systolic gradient decreased from mean of 51.4 ± 3.4 to 4.6 ± 0.7 mm Hg (P < 0.0001). There was one death 3 days postprocedure due to cerebral anoxia. There was one dissection diagnosed 24-hr postprocedure. At a mean follow-up of 45.9 ± 3.9 (3-120) months, all stents were patent and in good position on computed tomography. Four (7.1%) patients underwent successful redilation. Antihypertensive medication was decreased or stopped in 37 (66%) patients. CONCLUSIONS: Covered Cheetham-Platinum stents may be used effectively as therapy of choice in selected patients with severe coarctation of the aorta. Aortic wall complications occur even with covered stents. Covered stents provide a safe alternative to conventional stenting in the intermediate and long-term and can be redilated safely to keep pace with somatic growth.

Endovascular stenting in transverse aortic arch hypoplasia.

OBJECTIVES: Describe outcomes from stenting transverse aortic arch hypoplasia. BACKGROUND: Hypoplasia of the transverse arch may result in residual systemic hypertension and may be amenable to stenting. METHODS: Outcomes for transverse aortic arch hypoplasia stenting were collated from four centers between 2000 and 2010. Primary endpoints were reduction in peak systolic catheter gradient, dimensions of the stented segment, and systolic right arm blood pressure. Changes in antihypertensive medication and early and late complications were recorded. Data were collated for 21 patients (16 male, 5 female), median age of 16.5 years (range, 0.25-25.9 years) and median weight of 55 kg (range, 4.5-103 kg). 19/21 patients were hypertensive at baseline, excluding the two neonates after repair of interrupted aortic arch. RESULTS: Median transverse arch diameter increased from 7 to 14 mm after stenting (P < 0.001). Median ratio of the transverse arch to descending aorta at the diaphragm level improved from 0.43 to 0.9 (P < 0.001). Mean gradient across the hypoplastic transverse arch was 38 mm Hg (range, 14-76) at baseline and 5 mm Hg (range, 0-13) after stenting (P < 0.001). There were no deaths and 6 early complications occurred in 5 patients. Follow-up (median 24 months) data were available for 19 patients. 17/19 hypertensive patients had follow-up data. Two neonates developed intimal hyperplasia within the stent with a stent fracture in one. Median systolic blood pressure was 153 mm Hg (range, 117-180) prestent and 130 mm Hg (range, 105-150) poststent (P = 0.0002). In 13/17 patients, the antihypertensive medication could be reduced. CONCLUSIONS: Stenting of transverse aortic arch hypoplasia, although technically challenging, produced good angiographic and haemodynamic results with an early improvement in blood pressure control. These results appear to be sustained in the medium term.

Clinical and epidemiological features of pediatric population hospitalized with COVID-19: a multicenter longitudinal study (March 2020-December 2021) from Pakistan.

Background: We aimed to explore the epidemiological, clinical, and phenotypic parameters of pediatric patients hospitalized with COVID-19 in Pakistan. Methods: This longitudinal cohort study was conducted in five tertiary care hospitals in Pakistan from March 2020 to December 2021. Data on various epidemiological and clinical variables were collected using Case Report Forms (CRFs) adapted from the WHO COVID-19 clinical data platform at baseline and at monthly follow-ups for 3 months. Findings: A total of 1090 children were included. The median age was 5 years (Interquartile range 1-10), and the majority presented due to new signs/symptoms associated with COVID-19 (57.8%; n = 631), the most common being general and respiratory symptoms. Comorbidities were present in 417 (38.3%) children. Acute COVID-19 alone was found in 932 (85.5%) children, 81 (7.4%) had multisystem inflammatory syndrome (MIS-C), 77 (7.0%) had overlapping features of acute COVID-19 and MIS-C, and severe disease was found in 775/1086 (71.4%). Steroids were given to 351 (32.2%) patients while 77 (7.1%) children received intravenous immunoglobulins. Intensive care unit (ICU) care was required in 334 (31.6%) patients, and 203 (18.3%) deaths were reported during the study period. The largest spike in cases and mortality was from July to September 2021 when the Delta variant first emerged. During the first and second follow-ups, 37 and 10 children expired respectively, and medical care after discharge was required in 204 (25.4%), 94 (16.6%), and 70 (13.7%) children respectively during each monthly follow-up. Interpretation: Our study highlights that acute COVID-19 was the major phenotype associated with high severity and mortality in children in Pakistan in contrast to what has been observed globally. Funding: The study was supported by the World Health Organization (WHO), which was involved in the study design but played no role in its analysis, writeup, or publication.

Device closure of atrial septal defect: medium-term outcome with special reference to complications.

BACKGROUND: There are concerns over the outcome of device closure of secundum atrial septal defect with special reference to erosions and aortic regurgitation. AIM: To assess the medium-term outcome of device closure of atrial septal defects with special reference to complications. METHODS: A total of 205 patients with secundum atrial septal defects underwent transcatheter closure from October, 1999 to April, 2009. The median age was 18 (1.4-55) years. Amplatzer Septal Occluder was used in all the patients. Medium-term follow-up was available in 176 of 200 (88%) patients. RESULTS: Device closure was successful in 200 out of 205 (98%) patients. The device embolised in four patients and was associated with short inferior caval vein margin (p = 0.003). Balloon sizing in 71 patients (35%) resulted in implantation of a larger device (p = 0.002). Early complications included pericardial effusion, 2:1 heart block, and infective endocarditis (1 patient each). There were eight patients who reported migraine (3.9%). At median follow-up of 5.8 (0.6-10.3) years, complete closure occurred in 197 out of 200 patients. Short superior caval vein margin was associated with a residual shunt (p < 0.001). There were two patients who developed mild aortic regurgitation (1%), which correlated with a device-to-defect ratio of >1.3:1 (p = 0.001). There were no erosions, late embolisation, or thromboembolism. Atrial fibrillation occurred in three adults (1.5%). CONCLUSIONS: Device closure of secundum atrial septal defects using Amplatzer Septal Occluder is safe and effective in the medium term. Short inferior caval vein margin correlates with increased risk of embolisation and short superior caval vein margin with a residual shunt. The risk of developing aortic regurgitation is low and correlates with increased device-to-defect ratio.

Association of Blood Groups with the Severity and Outcome of COVID-19 Infection in Children.

The objective of this study was to find out the association of ABO blood groups with the severity and outcome of corona virus disease 2019 (COVID-19) in children. It included all laboratory-confirmed cases of COVID-19 and post-COVID multisystem inflammatory syndrome in children (MIS-C)/ Kawasaki disease (KD) like illness, admitted from March to September, 2020 to The Children's Hospital, Lahore. Out of 66 children, 45 (68.2%) were COVID-19 and 21 (31.8%) MIS-C/KD temporally associated with SARS-C0V-2. The mean age was 7.9 ± 4.2 years. Majority of children had mild to moderate illness 38 (57.6%), while 23 (34.8%) had severe or critical disease. Among all patients, 24 (36.4%) had some underlying comorbidity. Blood group A was significantly associated with severe and critical disease (p=0.030). COVID-19 in children had generally a good outcome, but children with blood group A were more susceptible to severe/critical disease. Key Words: Coronavirus disease 2019, ABO blood groups, Children, Severity, Outcome.

Association of Blood Groups with the Severity and Outcome of COVID-19 Infection in Children.

The objective of this study was to find out the association of ABO blood groups with the severity and outcome of corona virus disease 2019 (COVID-19) in children. It included all laboratory-confirmed cases of COVID-19 and post-COVID multisystem inflammatory syndrome in children (MIS-C)/ Kawasaki disease (KD) like illness, admitted from March to September, 2020 to The Children's Hospital, Lahore. Out of 66 children, 45 (68.2%) were COVID-19 and 21 (31.8%) MIS-C/KD temporally associated with SARS-C0V-2. The mean age was 7.9 ± 4.2 years. Majority of children had mild to moderate illness 38 (57.6%), while 23 (34.8%) had severe or critical disease. Among all patients, 24 (36.4%) had some underlying comorbidity. Blood group A was significantly associated with severe and critical disease (p=0.030). COVID-19 in children had generally a good outcome, but children with blood group A were more susceptible to severe/critical disease. Key Words: Coronavirus disease 2019, ABO blood groups, Children, Severity, Outcome.

Infection Prevention and Control Measures at the Children Hospital Lahore: A My Child Matters Collaborative Project.

PURPOSE: Infection prevention among children with cancer is a major challenge at Children Hospital Lahore (CHL), a public health care facility in Pakistan with 1,000 new pediatric cancer admissions annually. The objective has been to reduce infections through collaboration between CHL and the St Jude Children's Hospital Global Infectious Disease program via a grant by the Sanofi Espoir foundation through the My Child Matters program. The aim of the current study was to describe the effect of the collaborative improvement strategy on existing infection prevention and control (IPC) standards at CHL. MATERIALS AND METHODS: Our work was a prospective before-and-after study to improve IPC standards. We compared the WHO Hand Hygiene Self-Assessment Framework and four modules of the St Jude modified Infection Control Assessment Tool (ICAT) scores over a 3-year period. Our strategy included creating a multidisciplinary team of pediatric oncologists, infectious disease physicians, nurses, a microbiologist, and a data manager; engaging in monthly online IPC mentoring sessions with St Jude Children's Hospital Global Infectious Disease program and My Child Matters mentors; performing daily inpatient health care-associated infection surveillance rounds; and performing regular hand hygiene training and compliance audits. RESULTS: Baseline needs assessment showed health care-associated infections identified by positive blood cultures as 8.7 infections per 1,000 patient-days. Deficient hand hygiene supplies, health education measures, and bed sharing of neutropenic patients were identified as major challenges. Our hand hygiene facility level, per WHO scores, increased from Inadequate to Intermediate/Consolidation by the end of the 3-year implementation (122 v 352 WHO Hand Hygiene Self-Assessment Framework scores). The sink:bed and hand sanitizer:bed ratios improved to 1:6 and 1:1, respectively. The ICAT general infection control module increased by 40% (45 v 78 ICAT scores) and hygiene compliance improved by 20%. CONCLUSION: Implementing a collaborative improvement strategy improved IPC standards in our center, which can be easily replicated in other pediatric oncology centers in lower- and middle-income countries.

Pattern of ventricular septal defects and associated complications.

OBJECTIVE: To determine the frequency of various types of Ventricular Septal Defect (VSD) in local population and their complications. STUDY DESIGN: Case series. PLACE AND DURATION OF STUDY: The Paediatric Cardiac Unit of the Children Hospital and Institute of Child Health, Lahore, from January 2006 to December 2007. METHODOLOGY: 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. RESULTS: 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. CONCLUSION: 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.

OBJECTIVE: To determine pulmonary artery variations and other associated cardiac defects in patients with Tetralogy of Fallot (TOF). STUDY DESIGN: Cross-sectional, descriptive study. PLACE AND DURATION OF STUDY: The Children's Hospital and the Institute of Child Health, Lahore, from April 2006 to October 2007. METHODOLOGY: 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. RESULTS: 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. CONCLUSION: 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.

Intermediate and long-term outcome of patients after device closure of ASD with special reference to complications.

BACKGROUND: Device closure of Secundum atrial septal defect (ASD) is an accepted mode of treatment in selected patients with a suitable defect. The major initial concern over the long-term outcome has been erosions and more recently development of aortic regurgitation. Objective was to assess the intermediate and long term outcome of patients with device closure of ASD with special reference to complications. METHODS: Two hundred and four patients with significant Secundum ASD, 16 months to 55 years (median 8 years) were considered for transcatheter closure with the Amplatzer septal occluder from October 1999 to April 2009 with follow up examinations at 1, 3, 6, and 12 months and thereafter at yearly interval. RESULTS: Device closure of ASD was done successfully in 202/204 patients. The immediate (first 24-hour) major complications included device embolization (n = 4), pericardial effusion (n = 1) and 2:1 heart block (n = 1). At a mean follow up of 4.9 years (90 days to 9.6 years, median 5.3 years) complete closure was documented in all patients. Two patients (1%) had developed mild aortic regurgitation. Atrial fibrillation occurred in 3 adult patients (1.5%) at a mean of 2 weeks post procedure with complete recovery within 6 months. There were no late embolizations, erosions or thromboembolic events on long-term follow up. CONCLUSIONS: Device closure of Secundum ASD using Amplatzer septal occluder is safe and effective in intermediate and long-term follow up with extremely low mortality rate. The risk of development of aortic regurgitation or atrial fibrillation is also very low.

Congenital heart disease and thyroid dysfunction in Down syndrome reported at Children`s Hospital, Lahore, Pakistan.

Yaqoob M, Manzoor J, Hyder SN, Sadiq M. Congenital heart disease and thyroid dysfunction in Down syndrome reported at Children`s Hospital, Lahore, Pakistan. Turk J Pediatr 2019; 61: 915-924. Down syndrome is one of the main causes of intellectual disability in children. It occurs in every community and ethnic group. Several co-morbid conditions are associated with this syndrome. The present study was conducted to determine the frequency of congenital heart disease and thyroid dysfunction in a group of children affected with this genetic disorder. It was a record based retrospective study. A child having specific clinical features and chromosomal analysis showing an extra chromosome 21 was diagnosed as having Down syndrome. Diagnosis of congenital heart disease was based upon results of echocardiography. Thyroid dysfunction was diagnosed when either or both tetraiodothyronine and thyroid-stimulating hormone serum levels were abnormal. Three hundred and fifty cases were enrolled in the study according to the preset criteria for Down syndrome. Two hundred and ten (60%) were males and 140 (40%) females showing male to female ratio as 1.5 to 1. Majority of the children (55.1%) presented between 7 months and 24 months of age. Congenital heart defects were found in 41.8% of the cases. Ventricular septal defect was the most common, 41%. Thyroid dysfunction was found in 22% (n=60) of the cases of which 5.9% (n=16) had hypothyroidism. In conclusion, the frequency of congenital heart disease and thyroid dysfunction in Down syndrome children is high. Early referral of these children to tertiary health care facilities is emphasized as timely detection and management of these comorbid conditions will help in reducing the morbidity and mortality in this group of children.