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Objective: To assess the impact of fetal echocardiography in altering the course of action for neonatal cardiac expertise and services available in Pakistan following an antenatal diagnosis of congenital heart defect (CHD). Study Design: Cross-sectional study. Place and Duration of Study: Pediatric Cardiology Unit, Armed Forces Institute of Cardiology & National Institute of Heart Diseases, Rawalpindi Pakistan, from Feb 2020 to Sep 2021. Methodology: Fetuses aged 18 to 38 weeks of gestational age referred to the Pediatric Cardiac Unit due to risk of or suspicion of congenital heart disease underwent fetal cardiac echo. Data were collected and reviewed retrospectively to seek any impact of the antenatal diagnosis on post-natal planning.Results: The mean age of the mothers was 29.79±5.24 years (range, 17-47 years), and the mean gestational age at which the fetal echocardiography examination was performed was 27.29±4.47weeks (range: 12–38 years). Of the 609 pregnancies, fetal echocardiogram evaluation was performed, and sixty-three (10.3%) fetal echocardiograms were abnormal. Of the 63, only 06(9.5%) returned to the neonatal cardiac unit for follow-up and treatment in the first ten days of life. Conclusion: The real impact of fetal echocardiography is possible only with the availability of well-developed, readily accessible fetal and neonatal cardiac services. Poverty and a struggling healthcare infrastructure confound the benefits.

Objective: To assess the immediate and short-term efficacy and safety of transcatheter closure of cardiac defects with Lifetech™ Konar-MFO in a Tertiary Cardiac Care Center. Study Design: Descriptive cross-sectional study. Setting and Duration of Study: Armed Forces Institute of Cardiology/National Institute of Heart Diseases, Rawalpindi Pakistan, from Mar 2019 to Dec 2021 Methodology: Patients of all ages and both gender undergone transcatheter closure of a cardiac defect with Konar-MFO from Jan 2021 to Dec 2021 were included in the study through non-probability consecutive sampling and approval by the institutional ethical review committee was taken prior to data collection. Data of patients undergone transcatheter closure with Konar-MFO from Mar 2019 to Dec 2020 was extracted retrospectively to assess the immediate and short-term efficacy and safety of the occluder. SPSS version-23 was used for data entry and analysis. Results: A total of 138 patients had transcatheter closure of a cardiac defect with Lifetech™ Konar-MFO during the study period. Case of VSD were 124(89.9%), PDA 9(6.5%), post-operative cases were 4(2.9%) along with 1(0.7%) case of coronary artery fistula (CAF) to the right ventricle. Out of 138 patients, 73(52.9%) were females while 65(47.1%) were males. The mean age was 9.3 ±8.1 years with a range of 6 months to 36 years. Mean fluoroscopy time was 13.68±9.74 min. The procedure was successful in 137(99.27%) cases. The device embolized in 2(1.45%) cases. Conclusion: In selected cases of VSD, PDA and in some post-operative cases the occlusion with Lifetech™ Konar-MFO is safe and efficacious with the added benefits of softness and versatility of the approach.

Recent increase in deployment of trans-catheter devices such as septal or ductal occluding coils and devices has been a possible risk factor for infective endocarditis, predominantly in the initial post deployment period before endothelialization has ensued. Though a long-term study of trans-catheter Atrial Septal Defect (ASD) closure revealed no cases of Infective Endocarditis, some case reports of endocarditis in trans-catheter device closure of ASD, VSD and Patent ductus arteriosus(PDA) advocate that residual defect after device occlusion may be a factor in the risk for Infective Endocarditis. We herein describe the case of a 9-month-old female patient who underwent device closure for peri-membranous ventricular septal defect (VSD) and developed fungal infective endocarditis in early post occlusion period which was confirmed on blood culture. Despite treatment with appropriate antifungal as per sensitivity, clinical condition of the patient deteriorated and it was decided to manage her with surgical explanation of device and closure of defect.

In congenital cardiac surgery, intracardiac repair procedures like ventricular septal defect (VSD), atrioventricular septal defect and tetralogy of fallot (TOF) are associated with conduction system injury. Fewer cases are reported describing life threatening complication of late post-operative complete heart block. We are reporting 2 patients who presented with late complete heart block after ventricular septal defect repair and managed by permanent pacemaker placement in AFIC Cath lab.

Patent ductus arteriosus (PDA) is a common cardiac anomaly making 5%-10 % of all congenital heart diseases. The incidence increases in premature, very low birth weight neonates. Transcatheter closure is a standard procedure for treating PDA.However, in pre-term and very low birth weight neonates it presents special problems thus becoming a limitation in performing the procedure. This case report describes a PDA closure in a 1.3 Kg infant in Armed Forces Institute of Cardiology,Rawalpindi.

Critical congenital pulmonary stenosis with intact interventricular septum is a cyanotic and potentially lethal neonatal cardiac anomaly. Percutaneous transcatheter balloon pulmonary valvuloplasty for critical pulmonary stenosis often presents technical difficulties due to supra-systemic pressure in right ventricle. Using transjugular approach instead of the femoral vein helps in dealing with this issue. We describe the successful use of right internal jugular vein approach in a case of critical pulmonary stenosis.

Atrial flutter (AFL) is a rare arrhythmia in neonatal and infantile period. Potentially, AFL might lead to severe morbidity and even a fatal outcome. However it can have a good prognosis with early diagnosis and prompt appropriate treatment. Synchronized electrical cardioversion or transoesophageal atrial overdrive pacing is recommended in both stable and unstable cases. Antiarrhythmic drugs can be tried in stable newborns though the sinus rhythm takes some time to return to normal.Excellent prognosis has been noted in newborns and infants having AFL after sinus rhythm has been restored. The risk of recurrence is low and prolonged antiarrhythmic therapy is less likely to be required. However follow-up after discharge is essential to look for any possibility of recurrent arrhythmia and possible untoward effects of treatment.

We report a technically difficult retrieval of embolized Atrial Septal Defect device from left atrium. Device was captured after multiple attempts using different types of snares and was brought to inferior vena cava without delivery sheath. As we were unable to slenderize the device into short sheath, it was finally retrieved using an unconventional and innovative technique.We emphasize the importance of safe improvised methods during challenging catheterization procedures resulting in successful outcomes.

Objective: To assess if adherence to international guidelines for pregnant cardiac patients’ mode of delivery is achievable with available local resources. Study Design: Prospective quasi experimental descriptive study. Place and Duration of Study: All Pregnant patients with cardiac disease reporting to Armed Forces Institute of Cardiac Disease/National Institute of Heart Disease (AFIC/NIHD) who were admitted for and were delivered from Oct 2009 to Sep 2011 were included in the study. Methodology: International guidelines and recommendations for mode of delivery for cardiac patients with pregnancy were followed for patients. Caesarean sections were reserved only for obstetrical reasons and absolute cardiac indications. The patients with first trimester miscarriage were not included. Data was collected on a personal computer Microsoft excel sheet. Frequency was calculated from the data entered. The reduction in caesarean section rate and increase in vaginal delivery rate as per guidelines without an increase in maternal mortality, was the main outcome. Results: Total of 221 patients were delivered in the 2 year period between October 2009 to September 2011 at AFIC/NIHD. The caesarean section rate the year before 2008-2009 had been 48.6%. After the change in practice by concurring to international guidelines, the caesarean section rate for the 2 year period dropped to 26.7%. The hospital maternal deaths were 5. This was slightly better to the year 2008-2009 which had 3 maternal deaths. Conclusion: It’s possible to adhere to international recommendations and protocols for pregnant cardiac patients and achieve internationally comparable outcome in terms of mode of delivery without increasing current institutional maternal mortality rate with local hospital resources.

After total correction for tetrolgy of fallot (TOF), right ventricle behaves in an unpredictable manner depending on type of right ventricular outflow tract (RVOT) reconstruction and surgical expertise of infundibular muscle resection. We are reporting a 23 years old girl who underwent total correction at two years of age. RVOT was reconstructed with native pericardial patch. Gradually she developed breathlessness and occasional chest pain. Echocardiograghy revealed hugely dilated right ventricle (RV) with gross pulmonary regurgitation and RV dysfunction. Cardiac MRI also calculated right ventricular end systolic volume (RVESV) 57 ml/m2 and right ventricular end diatolic volume (RVEDV) 157ml/m2.We decided to implant transcatheter venus p-valve at pulmonary position. The procedure went successful having competent pulmonary valve and improved RV function. Total fluoro time was 36.4 minutes and total procedural time was two hours. This procedure was done first time in Pakistan with optimal results.

Background: Tetralogy of Fallot (TOF) is characterized by ventricular septal defect, an overriding aorta, varying degrees of pulmonic stenosis, and right ventricular hypertrophy. It may be associated with a number of lesions including development of major aortopulmonary collateral arteries (MAPCAs) in less than 5% of cases. In this study we just focused on the management of MAPCAs. Methodology: TOF patients with significant MAPCAs were selected after cardiac catheterization and planned for transcatheter embolization just before surgery. Results: A total of 45 patients underwent transcatheter closure of MAPCAs just before surgical correction with 100% success rate. The age varied from 3-30 years and 55% (n=25) were female. In majority 60% (n=27) the procedure was carried out under deep sedation with local anaesthesia while 18 (40%) patients had general anaesthesia. There was insignificant residual leak in 2 (4%) patients, transient bradycardia (n=1) and hypercyanotic spell in 4% (n=2). No serious complications or death occurred during this study period. Conclusion: Transcatheter coiling of MAPCAs is safe, easy, and effective procedure and protects the patient from its long term devastating complications.

To analyze the effectiveness of transthoracic echocardiography (TTE) for device closure of secundum atrial septal defect in children ≤5 years old.Quasiexperimental study.The study was conducted at Armed Forces Institute of Cardiology and National Institute of Heart Diseases from December 1, 2010 to December 31, 2012.During the study period, a total of 48 children ≤5 years old underwent device closure of secundum atrial septal defect. The indications for closure were: elective closure in 31; parental anxiety in 10; frequent respiratory infection in 4; severe pulmonary stenosis in 2; and severe mitral stenosis in 1 patient. The procedure was carried out under general or local anesthesia with TTE and fluoroscopic guidance. TTE was the primary tool used for measurement of defect and estimation of occluder size as well as guiding equipment during device deployment in all patients.A total of 47/48 patients (97.9%) had successful closure of secundum atrial septal defect. The mean age was 4.1 ± .68 years (range, 2.5-5 years) and 28/48 patients (58.4%) were female. The defect size and occluders used were between 5-20 mm (mean, 12 ± 3.5 mm) and 8-22 mm (mean, 15 ± 3.9 mm), respectively. Three patients had simultaneous procedures comprising pulmonary balloon valvuloplasty in 2 patients and percutaneous transmitral commissurotomy in 1 patient. The device embolization occurred in 1 patient; the device was retrieved percutaneously and the patient was referred for surgical closure. The minor complications were residual leak (n = 1), transient bradycardia (n = 4), and first-degree heart block (n = 1). The median procedure time was 30 min (range, 15-100 min) and median fluoroscopic time was 6 min (range, 1.50-45 min). There were no emergency surgical explorations, cardiac perforations, vascular injuries, or deaths during this period.TTE can be used as a primary tool for the measurement of atrial septal defect and guidance during device deployment in young children by skilled and professional hands, yet more experience is awaited.

To determine the efficacy, safety and immediate complications encountered during percutaneous device closure of patent ductus arteriosus (PDA).Case series.Department of Paediatric Cardiology, AFIC/NIHD, Rawalpindi, from January 2005 to December 2010.Consecutive 500 patients who underwent attempted transcatheter PDA device closure were included in the study. Device type position, success of closure and complications were described as frequency percentage.In 491 cases (98.2%), PDA was successfully occluded including 4 cases (0.8%) where devices were dislodged but retrieved and redeployed in Cath laboratory. PDA occluder devices used in 448 cases (91%) while coils (single or multiple) were used in 42 cases (8.5%) and in one case (0.2%) ASD occluder device was used to occlude the PDA. There were 09 (1.8%) unsuccessful cases, 06 (1.2%) were abandoned as ducts were considered unsuitable for device closure, 02 (0.4%) devices dislodged and needed surgical retrieval and one case (0.2%) was abandoned due to faulty equipment. The narrowest PDA diameter ranged from 0.5-14 mm with mean of 4.5±2.4 mm. There was a single (0.2%) mortality.Transcatheter occlusion of PDA by coil or occluder device is an effective therapeutic option with high success rate. Complication rate is low in the hands of skilled operators yet paediatric cardiac surgical back-up cover is mandatory.

To evaluate the results and complications associated with transcatheter closure of patent ductus arteriosus (PDA) in infants.Quasi-experimental study.Paediatric Cardiology Department of Armed Forces Institute of Cardiology / National Institute of Heart Diseases (AFIC/NIHD), Rawalpindi, from December 2010 to June 2012.Infants undergoing transcatheter device closure of PDA were included. All patients were evaluated by experienced Paediatric Cardiologists with 2-D echocardiography and Doppler before the procedure. Success of closure and complications were recorded.The age of patients varied from 05 - 12 months and 31 (56.4%) were females. Out of the 55 infants, 3 (5.4%) were not offered device closure after aortogram (two large tubular type ducts and one tiny duct, considered unsuitable for device closure); while in 50 (96.1%) patients out of remaining 52, the duct was successfully closed with transcatheter PDA device or coil. In one infant, device deployment resulted in acquired coarctation, necessitating device retrieval by Snare followed by surgical duct interruption and another patient had non-fatal cardiac arrest during device deployment leading to abandonment of procedure and subsequent successful surgical interruption. Local vascular complications occurred in 12 (21.8%) of cases and all were satisfactorily treated.Transcatheter device closure of PDA in infants was an effective procedure in the majority of cases; however, here were considerable number of local access site vascular complications.

Pentalogy of Cantrell with ectopia cordis is a rare congenital anomaly, first described in 1958 by Cantrell, has a reported incidence of around 5-10 cases per one million live births with wide variety of clinical presentations. We are reporting a child with ectopia cordis along with cleft lower sternum, upper abdominal wall defect, ectopic umbilicus and diaphragmatic defect. Echocardiography in first month of life revealed a restrictive perimembranous ventricular septal defect and a small patent Foramen Ovale, both closed spontaneously in infancy. CT angiography at 10 months of age revealed a defect in the thoracic and abdominal walls along with herniation of left ventricular apex into epigastrium. The two ventriculi formed a tail that looked like a crocodile. This patient underwent surgical correction at our institution at 14 months of age and recovered well with no residual issue.

Tropical pyomyositis is a bacterial infection of the skeletal muscles leading to abscess formation, occurring in the tropical areas, often following minor trauma. We report a case of pancarditis as the direct complication of pyomyositis in a 10-year-old girl who presented with painful swelling of her right thigh, high grade fever and impaired consciousness. Echocardiography showed pericardial effusion with strands and a large vegetation in the left ventricle cavity. She was treated successfully with open heart surgical drainage and intravenous antibiotics. We emphasize early diagnosis and prompt treatment of pyomyositis to reduce its associated mortality and morbidities.

To assess the results of anterograde transcatheter closure of a large sized patent ductus arteriosus using the Amplatzer Duct Occluder (ADO) device.A case series.Department of Cardiology, Armed Forces Institute of Cardiology and National Institute of Heart Diseases, Rawalpindi, from January 2005 to December 2007.All patients who underwent transcatheter closure of large sized PDA (or = 4.0 mm), using the Amplatzer Duct Occluder (ADO) device, from January 2005 to December 2007, were included by convenient sampling technique. After the procedure, repeat aortogram was performed to confirm the appropriate position of the ADO and to evaluate for residual shunts.A total of 100 patients had PDA diameter ofor = 4.00 mm. Mean age was 11.73 + 10.31 years and there were 37 males and 63 females. Mean PDA diameter was 6.011 + 2.078 mm. Forty-nine patients had type 'A', 19 had type 'B', 19 patients had type 'C', 5 had type 'D' and 8 patients had type 'E' PDA. Complete closure was achieved in 70 patients, 26 had trace shunt and 4 patients had small residual shunt immediately after the procedure. However, no patient had residual shunt 24 hours after the procedure.ADO is a highly efficient prosthesis that can be safely applied in most patients with PDA particularly in patients with large sized PDA.

Lutembacher's syndrome is a rare combination of atrial septal defect (ASD) and rheumatic mitral stenosis. Traditionally, this condition is treated surgically. We present a case of Lutembacher's syndrome that was successfully treated with percutaneous transcatheter mitral commissurotomy (PTMC) using the Inoue balloon. Closure of ASD was successfully achieved with an Amplatzer atrial septal occluder under transthoracic echocardiogram (TTE) guidance without general anesthesia.