Transcatheter Palliation of a Young Infant With Obstructed Supracardiac Total Anomalous Pulmonary Venous Connection.

Obstructed total anomalous pulmonary venous connection is a life-threatening pediatric cardiac emergency. Infants usually present in critical condition with marked respiratory distress, severe metabolic acidosis, and central cyanosis. Urgent cardiac surgical intervention, despite its high risk, is necessary in order to save the life of the patient. A two-month-old female infant presented to our tertiary care hospital with dense cyanosis and metabolic acidosis. She required mechanical ventilation, but her oxygen saturation did not improve. Her 2D transthoracic echocardiography revealed obstructed supracardiac total anomalous pulmonary venous connection with adequate interatrial communication and severe pulmonary hypertension. After discussion with the family and pediatric cardiac surgical team, it was decided to offer her transcatheter relief of obstructive ascending channel. She underwent successful balloon angioplasty of stenosed levoatrial cardinal vein (vertical vein) with remarkable improvement in blood flow and vessel caliber. She was extubated and her oxygen saturation rose from the high seventies to low eighties immediately after the procedure. She is scheduled for cardiac surgical repair within the next few days. Transcatheter angioplasty is a workable option in stabilizing very sick young infants with obstructed total anomalous pulmonary venous connection, especially supracardiac ones.

Quadricuspid aortic valve and a large patent ductus arteriosus treated with device closure.

Quadricuspid aortic valve is a remarkably rare congenital cardiac anomaly that predominantly becomes regurgitant with the passage of time. Aortic valve stenosis and aortic root dilatation are less common in quadricuspid aortic valve as compared to bicuspid aortic valve. The clinical presentation depends upon the functional status of the aortic valve, left ventricular function, and associated cardiac or coronary anomalies. The quadricuspid aortic valve is easily visualised during transthoracic echocardiogram with a characteristic X pattern of aortic valve in diastole. The association of quadricuspid aortic valve with patent ductus arteriosus is exceedingly rare. We are reporting a case of young girl with mildly regurgitant quadricuspid aortic valve, large patent ductus arteriosus, and volume-loaded left heart who underwent a successful device closure of her patent ductus arteriosus. To the best of our knowledge, such a case is being reported from Pakistan for the first time.

Bidirectional Glenn for residual outflow obstruction in Tetralogy of Fallot.

BACKGROUND: Residual right ventricular outflow obstruction during Tetralogy of Fallot repair necessitates peri-operative revision often requiring trans-annular patch with its negative sequels. Bidirectional Glenn shunt in this setting reduces trans-pulmonary gradient to avoid revision. METHODS: Bidirectional Glenn shunt was added during Tetralogy repair in patients with significant residual obstruction. A total of 53 patients between January, 2011 and June, 2018 were included. Final follow-up was conducted in July, 2018. RESULTS: Mean age at operation was 5.63±3.1 years. Right to left ventricular pressure ratio reduced significantly (0.91±0.09 versus 0.68±0.05; p<0.001) after bidirectional Glenn, avoiding revision in all cases. Glenn pressures at ICU admission decreased significantly by the time of ICU discharge (16.7±3.02 versus 13.5±2.19; p<0.001). Pleural drainage ≥ 7 days was seen in 14 (26.4%) patients. No side effects related to bidirectional Glenn-like facial swelling or veno-venous collaterals were noted. Mortality was 3.7%. Discharge echocardiography showed a mean trans-pulmonary gradient of 32.11±5.62 mmHg that decreased significantly to 25.64±5 (p<0.001) at the time of follow-up. Pulmonary insufficiency was none to mild in 45 (88.2%) and moderate in 6 (11.8%). Mean follow-up was 36.12±25.15 months (range 0.5-90). There was no interim intervention or death. At follow-up, all the patients were in NYHA functional class 1 with no increase in severity of pulmonary insufficiency. CONCLUSION: Supplementary bidirectional Glenn shunt significantly reduced residual right ventricular outflow obstruction during Tetralogy of Fallot repair avoiding revision with satisfactory early and mid-term results.

Transcatheter device closure of patent ductus arteriosus.

OBJECTIVE: To determine the efficacy, safety and immediate complications encountered during percutaneous device closure of patent ductus arteriosus (PDA). STUDY DESIGN: Case series. PLACE AND DURATION OF STUDY: Department of Paediatric Cardiology, AFIC/NIHD, Rawalpindi, from January 2005 to December 2010. METHODOLOGY: 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. RESULTS: 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. CONCLUSION: 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.

Percutaneous transcatheter PDA device closure in infancy.

OBJECTIVE: To evaluate the results and complications associated with transcatheter closure of patent ductus arteriosus (PDA) in infants. STUDY DESIGN: Quasi-experimental study. PLACE AND DURATION OF STUDY: Paediatric Cardiology Department of Armed Forces Institute of Cardiology / National Institute of Heart Diseases (AFIC/NIHD), Rawalpindi, from December 2010 to June 2012. METHODOLOGY: 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. RESULTS: 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. CONCLUSION: 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.

Safety and technical success of percutaneous left main coronary artery stenting.

OBJECTIVE: Critical stenosis of left main coronary artery (LMCA) has always remained a challenge for interventional cardiologists. Conventionally Coronary Artery Bypass Grafting (CABG) is done for these patients but recently Percutaneous Coronary Intervention (PCI) is also being tried more frequently, but data of PCI is scarce in this regard. Our objective was to determine the safety and technical success rate of percutaneous left main coronary artery stenting. METHODS: This was 12 month follow up study conducted at Pakistan Institute of Medical Sciences (PIMS), Islamabad from 11(th) Jan 2012 to 11(th) Jan 2013. All symptomatic patients who underwent coronary angiogram at PIMS and were found to have either isolated LMCA disease or coexisting osteal Left Anterior Descending (LAD) artery disease were potentially eligible for the study. Patients who had previous surgical treatment for coronary artery disease and those with renal dysfunction requiring dialysis were excluded. Patients were counselled in detail regarding the pros and cons of PCI versus CABG.Those who opted for PCI were included in the study. All these patients were treated with percutaneous left main coronary artery stenting with or without osteal LAD stenting. RESULTS: Seventy two patients had LMCA disease during angiogram. Fifteen patients opted for CABG. Four patients did not meet the inclusion criteria, whereas 53 patients were finally enrolled. Mean age of patients were 55.45±10.275 years. Twenty nine patients were with acute coronary syndrome and 22 presented with unstable angina.PCI with stenting was technically successful in all patients. One patient died three months after PCI, there was no other mortality. CONCLUSION: Our study showed that Percutaneous Coronary Intervention (PCI) to LMS has good technical success rate; the safety of the procedure is also acceptable.

Transthoracic echocardiography as a measuring and guiding tool for transcatheter device closure of secundum atrial septal defect in young children.

OBJECTIVE: To analyze the effectiveness of transthoracic echocardiography (TTE) for device closure of secundum atrial septal defect in children ≤5 years old. STUDY DESIGN: Quasiexperimental study. STUDY LOCATION AND DURATION: The study was conducted at Armed Forces Institute of Cardiology and National Institute of Heart Diseases from December 1, 2010 to December 31, 2012. PATIENTS AND METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Successful surgical repair of pentalogy of cantrell at 14 months of age.

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.

Device closure of atrial septal defect with amplatzer septal occluder in adults- safety and outcome.

BACKGROUND: Secundum atrial septal defect is a common congenital heart disease. Amplatzer septal occluder has become the most commonly used device for its closure. This study was conducted to determine the safety and outcome of atrial septal defect closure with the Amplatzer septal occlude (ASO). METHODS: This Case-series was conducted at Armed Forces Institute of Cardiology/National Institute of Heart Diseases Rawalpindi from July 2010-11, on a total of 41 patients, out of these 41 patients, 18 (44%) patients underwent general anaesthesia while 23 (56%) patients were given local anaesthesia for the procedure. Trans-esophageal echocardiogram was done in all patients before procedure. RESULTS: In 37 patients, ASD device closure was successfully done and size of ASD devices were 14-36 mm. Trans-oesophageal echocardiography guided ASD Device closure was done in 16 patients and rest of them done under transthoracic echocardiography. Size of ASD ranged from 11-36 mm (mean 22 mm). Mean floro time was 10 minutes. Four (10%) patients did not undergo the procedure due to inadequate rim. CONCLUSION: Device closure of Atrial Septal Defect with amplatzer septal occluder is convenient and safe.

Pancarditis: a rare complication of tropical pyomyositis.

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.

Percutaneous transluminal mitral commissurotomy for rheumatic mitral stenosis in a 5-year-old child.

We report a 5-year-old boy weighing 11 kg, with severe mitral valve stenosis of rheumatic aetiology, who underwent successful percutaneous transluminal mitral commissurotomy (PTMC) with valvuloplasty balloon. Postprocedural mean pressure gradient across the mitral valve decreased to 6 mmHg from an initially recorded value of 22 mmHg. In addition to symptomatic improvement, the mitral valvular area increased from 0.4 to 0.8 cm(2) without significant change in mitral regurgitation. At 1- and 3-month follow up, transthoracic echocardiography revealed further improvement with an increase in mitral valve area to 1.0 cm(2), a decrease in pulmonary arterial pressure, and a mean mitral valve pressure gradient of 8 mmHg with trivial mitral regurgitation. To best of our knowledge, this is the first successful PTMC procedure performed in the youngest and smallest ever reported child with rheumatic mitral stenosis (MS). We conclude that PTMC with valvuloplasty balloon could be a logical alternative to surgery in young patients with rheumatic MS.

Comparative study of efficacy, tolerability and compliance of oral iron preparations (iron edetae, iron polymatose complex) and intramuscular iron sorbitol in iron deficiency anaemia in children.

OBJECTIVE: To compare the efficacy, tolerability and compliance of oral iron preparations (iron edetate and iron polymaltose complex) with each other and with intramuscular iron sorbitol in iron deficiency anaemia in children. METHODS: A Randomized Controlled Trial (RCT) was carried out at the Paediatric Department of Combined Military Hospital (CMH) from January 2006 to December 2007. In total 146 children, up to 12 years age having haemoglobin (Hb%) less than 8 gm% were included. They were randomly distributed into three groups. Group A (64 cases) received oral sodium iron edetate (SIE), Group B (40 cases) received oral iron polymaltose complex (IPC) and group C (42cases) received intramuscular iron sorbitol (IS) in recommended dosages. Rise in Hb% > 10gm% was kept as desired target. Maximum duration of treatment planned was 2 weeks for parenteral iron (group C) and 12 weeks for oral iron (groups A and B). Haematological parameters- Hb%, mean corpuscular volum (MCV), mean corpuscuar haemoglobin (MCH), mean corpuscular haemoglobin concentration (MCHC) were measured at induction followed at 2 weeks, 4 weeks, 8 weeks and 12 weeks after start of treatment. Compliance and drop out rates were determined on each visit. Data was analyzed using SPSS version10.ANOVA was used to analyze difference in rise in Hb% at various intervals. RESULTS: Statistically significant increase in mean Hb%, MCV, MCHC after 02 weeks was observed in group C (IS). Rise in these parametes became significant in group A (SIE) and B (IPC) after 04 weeks. Peristent rise was observed in oral groups at 08 and 12 weeks. Rise in Hb% was much faster in group C (IS). It took 2 weeks to achieve mean Hb% > 10gm% and compliance rate was 40.5%, while to achieve same target, duration required was 8 weeks in group A (SIE) and 12 weeks in group B (IPC) and compliance rate was 39% and 30% respectively. Adverse effects were much more common with group A (SIE) as compared to other two groups. CONCLUSION: Intramuscular iron sorbitol is a reliable and faster alternative modality for treatment of iron deficiency anaemia in children. Short duration of treatment, sure rise in Hb% and minimal adverse effects improve compliance as compared to oral preparations. Among oral preparations, rise in Hb% is more rapid with iron edetae. While IPC gives relatively slower rise in Hb% but side effects are much less as compared to SIE.

Etiology of short stature in children.

OBJECTIVE: To determine the causes of short stature in children with special emphasis on growth hormone deficiency. STUDY DESIGN: Case series. PLACE AND DURATION OF STUDY: Department of Paediatrics, Military Hospital, Rawalpindi and Combined Military Hospital, Multan from September 2004 to January 2007. METHODOLOGY: Two hundred and fourteen children (140 boys and 74 girls), ranging from 02 to 15 years presenting with short stature were studied. Height and weight were plotted on appropriate growth charts and centiles determined. Relevant hematological and biochemical investigations including thyroid profile were done. Bone age was determined in all cases. Growth hormone axis was investigated after excluding other causes. Karyotyping was done in selected cases. Data was analyzed by SPSS 10.0 by descriptive statistics. Mean values were compared using t-test. RESULTS: In this study, the five most common etiological factors in order of frequency were Constitutional Growth Delay (CGD), Familial Short Stature (FSS), malnutrition, coeliac disease and Growth Hormone Deficiency (GHD). In 37.4% of patients, the study revealed normal variants of growth - CGD, FSS or combination of both, 46.7% cases had nonendocrinological and 15.9% had endocrinological etiology. CGD (22.1%) in males and FSS (27%) in females were the most common etiology. GHD was found in 6.1% children and it comprised 38.2% of all endocrinological causes. Children with height falling below 0.4th centile were more likely to have a pathological short stature (79.2%) compared to 39.3% whose height was below 3rd centile but above 0.4th centile (p < 0.05). CONCLUSION: CGD and FSS are most common causes of short stature in boys and girls respectively, whereas, GHD is a relatively uncommon etiology.

Cerebellar tuberculoma mimicking posterior cranial fossa tumour.

A young girl presented with history of prolonged fever, vomiting and headache. CT scan of brain revealed a space occupying lesion in posterior cranial fossa with moderate hydrocephalus. Surgery was performed and histopathology report confirmed the lesion as tuberculous. Patient showed smooth postoperative recovery and complete remission of complaints on antituberculous treatment for one year and regular follow-up.

Breast feeding patterns in a military hospital.

OBJECTIVE: To determine the status of breast feeding, exclusive breast feeding and other feeding patterns in infants upto 12 months age. DESIGN: Cross- sectional study. PLACE AND DURATION OF STUDY: Paediatrics Department and Immunization Center, Combined Military Hospital, Multan, from October 2004 to March 2005. SUBJECTS AND METHODS: This study was based on "current status analysis method" to eliminate the bias of recall method used in most previous studies. Mothers of 650 infants from 0-12 months of age, attending immunization centre and paediatrics OPD, were interviewed about current feeding patterns and other socioeconomic variables. They were divided in three groups depending upon their monthly income. Living conditions matched with monthly income status in all groups. Month-wise distribution of feeding pattern was determined. Analysis was made about the effect of education of mothers, family income, place and mode of delivery, gender of the baby on exclusive breast feeding, duration of breast feeding and initiation of breast feeding. Statistical analysis of the results was made by application of Parson's Chi-square test. RESULTS: Breast feeding was maintained at high level (more than 88%) throughout infancy. Exclusive breast feeding was also maintained at quite high level (66%) till 4 month age. But later there was a sharp decline to 16% at six months of age. The median duration of exclusive breast feeding was 3 months. Mothers with lesser education and lower family income were more likely to exclusively breast feed (p < 0.001). The time interval between birth and first feed observed was quite desirable (less than 3 hours) in most cases. Only 14% infants received pre-lacteal feeds (ghutti, honey, water etc.), while majority of them (59%) received breast milk. Formula milk was first feed in only 25% cases. Breast milk as first and early feed was observed more in babies born by normal delivery in hospital (p < 0.001). CONCLUSION: Status of breast feeding, exclusive breast feeding and other feeding practices was quite encouraging and desirable in this study. The factors responsible for these results are comprehensive, free and readily available medical facilities including motivational and preventive services provided to women and children in military hospitals. However, there is still need to improve the figures to achieve internationally set goals.

Assessment of a new approach to family planning services in rural Pakistan.

OBJECTIVES: In 1993, the government of Pakistan started a new approach to the delivery of contraceptive services by training literate married women to provide doorstep advice and supplies in their own and neighboring communities. This report assesses whether this community-based approach is starting to have an impact on contraceptive use in rural areas. METHODS: A clustered nationally representative survey was used to collect data on contraceptive use and access to services in each cluster. Two-level logistic regression was applied to assess the effects of service access, after potential confounders were taken into account. RESULTS: Married women living within 5 km of 2 community-based workers were significantly more likely to be using a modern, reversible method of contraception than those with no access (odds ratio = 1.74; 95% confidence interval = 1.11, 2.71). CONCLUSIONS: After decades of failure, the managers of the family planning program have designed a way of presenting modern contraceptives that is appropriate to the conditions of rural Pakistan. The new community-based approach should be steadily expanded.