Placement of permanent pacemaker in a low-birth-weight infant with congenital heart block: a case report.

One of the rare diseases with a high mortality rate in infants is congenital heart block (CHB) with neonatal lupus erythematosus (NLE) as the most common cause. A permanent pacemaker (PPM) is indicated for symptomatic bradycardia. The choice of PPM in the paediatric population is different from that in the adult population because of several reasons like small size, account of somatic growth, and difference in physiological changes. Here, we present a case in which a 2.6 kg and 45 days old baby with CHB secondary to NLE was successfully treated with a single-chambered adult-sized PPM with epicardial lead. According to our knowledge, this is the smallest baby in Pakistan in which PPM has been implanted.

Anomalous left coronary artery from pulmonary artery (ALCAPA) as a silent cause of mitral regurgitation in children.

Anomalous left coronary artery from pulmonary artery (ALCAPA), also known as Bland-White-Garland syndrome, is a rare cardiac disease. This condition may present with complications such as myocardial infarction, left ventricular dilatation, mitral regurgitation, and left heart failure in children. We report a case of a four-year-old boy who presented with shortness of breath, palpitations, and recurrent upper respiratory tract infections. He was diagnosed with mitral regurgitation. During the surgery, left coronary artery (LCA) was not present in its anatomical position and ALCAPA was identified. One should keep in mind the possibility of ALCAPA in presentation of mitral regurgitation in children despite not being reported in echocardiography.

Comparison of figure of eight and traditional simple wire closure method to prevent dehiscence after sternal closure.

OBJECTIVE: To investigate significant differences, if any, between figure of-eight method and simple wire closure technique in reducing the incidence of infectious and non-infectious sternal dehiscence in patients undergoing sternal closure. METHODS: The systematic review was conducted in the Cardiothoracic Surgery Department at Aga Khan University from 1st December 2015 to 13th December 2017. The review was registered with PROSPERO, the International Prospective Register of Systematic Reviews. Pubmed, Cochrane Library (Wiley) and Scopus databases were searched for articles published up to December 13, 2017. The search was limited to randomised control trials, clinical trials, retrospective cohort studies, journal analyses, systematic reviews and meta analyses. Cadaveric or animal studies and articles published in languages other than English were excluded. RESULTS: Of the 286 articles retrieved, 265(92.6%) were excluded on the basis of study title and abstract. Another 15(5.2%) were excluded for being irrelevant to the topic n hand, and 6(2.1%) formed the final sample. Of them, 4(66.6%) studies showed no significant difference between the two techniques, while 2(33.3%) found figure-of-eight technique to be superior of the two. CONCLUSIONS: There was no significant difference between the figure-of eight method and the simple wire technique in reducing the risk of dehiscence in patients undergoing sternal closure.

Adult congenital cardiac life-long needs evaluation in a low-middle income country, Pakistan.

OBJECTIVE: Adult congenital heart diseases (ACHD) have distinct health care needs that require life-long care. Limited data is available from low-middle income countries (LMIC). This descriptive study conducted in Pakistan, aimed to assess patients and health care professionals understanding of the needs for ACHD care and the perceived barriers to care. METHODS: A telephone survey was conducted of ACHD patients. An e mail survey was sent to the paediatric and adult cardiologists of five institutions (3 public and 2 private) that provide ACHD services in Pakistan. Descriptive statistics (frequencies, mean ± SD, median) were used for data analysis. RESULTS: A total of 128 ACHD patients were surveyed, 65 (51%) were females with a mean age of 29.4±10.4 years. Atrial septal defect repair was the most common surgical procedure. Mean age at surgery was 25.6±10.49 years, and a surgical follow-up period of 3.8±2.3 years. Majority (n=3, 60%) of the health care professionals (HCPs) responded that 75-100% of the ACHD surgical patients would need lifelong care, yet 10-25% return to their cardiology clinics. Most of the surveyed ACHD patients (89%, n=114) demonstrated a lack of understanding of life-long care after surgery due to not being communicated by their HCPs. Cost and travelling issues were the barriers highlighted by HCPs. Both ACHD patients (96%, n=122) and HCP (100%, n=5) underscored their interest in life long care. CONCLUSIONS: Majority of ACHD patients in Pakistan did not know that life-long follow-up is needed. Education regarding lifelong care for ACHD patients was identified as a means to alleviate the knowledge gap.

Major Septal Defects: Comparative Study of Down Syndrome and Non-Down Syndrome Infants, Before and After Surgery.

OBJECTIVE: To compare pre-operative, intra-operative, and post-operative parameters in Down syndrome (DS) and non-DS patients with atrioventricular septal defects (AVSD) and inlet ventricular septal defects (VSD) in a tertiary care hospital in Pakistan. METHODS: We conducted a retrospective study at Aga Khan University, Pakistan. All complete atrioventricular septal defect (CAVSD), partial atrioventricular septal defect (PAVSD), and VSD with inlet extension surgical cases from January 2007 to January 2019 were included. Patients with congenital heart diseases other than those listed above were excluded. RESULTS: In 61 cases, 18 had DS. Median age, mean body surface area (BSA), and height were lower in DS patients compared to non-DS patients: 7.0 vs 23.0 months, 0.311 vs 0.487 m2, and 63 vs 82 cm, respectively. Bypass duration, aortic cross clamp time, post-operative ventilator hours, dose of inotropes, CICU stay, and total hospital stay were all significantly higher in the DS group. The odds ratio (955% CI) for mortality in DS babies was 6.2 (1.4, 27.1), p=0.015, after adjusting for age, weight, and height. The overall morbidity was comparable between the two groups, demonstrating no significant difference after adjusting for confounders. CONCLUSION: DS babies with AVSD and inlet VSD are at a greater risk of mortality compared to non-DS babies, particularly those with CAVSD. Furthermore, DS babies undergo surgery at a younger age and require more aggressive post-operative therapy and monitoring due to the development of complications.

Minimally invasive technique of placing a dual chamber permanent pacemaker in children.

OBJECTIVE: To share the experience of a minimally invasive technique in the implantation of a dual chamber permanent pacemaker in paediatric population. METHODS: The retrospective study was conducted at Aga Khan University Hospital, Karachi, and comprised data of patients aged up to 16 years who underwent epicardial dual chamber permanent pacemaker insertion via xiphisternal incision between April 2011 and August 2016. Demographic data included age, weight and gender of the patient. Indications for pacemaker insertion, electrocardiography findings, concomitant cardiac procedures and procedural complications were reviewed. Pacemaker thresholds and impedance at the time of implantation and throughout the course of follow-up were extracted from the clinical data. RESULTS: Of the 10 patients, 5(50%) were males and 5(50%)were females. The overall mean age was 3.4}3.8 years (range: 1 month - 13 years). The mean weight at the time of operation was 11.4}6.8 kg (range: 4.3-27kg). Indications for permanent pacemaker insertion included postoperative advanced or complete atrioventricular block in 7(70%) and complete congenital heart block in 3(30%). There was no reported morbidity. CONCLUSIONS: Dual chamber permanent pacemaker insertion via xiphisternal incision was found to be of benefit to the patients and the surgeons alike.

Sinus of valsalva aneurysm rupturing into main pulmonary artery: A rare paediatric cardiac emergency.

Congenital Sinus of Valsalva aneurysm is a rare congenital cardiac disease. Most common site of origin is the right sinus. It ruptures into right ventricle or right atrium most of the times. Only in less than 2% of the cases it ruptures into the pulmonary artery. We report a rare case of right sinus of valsalva aneurysm rupturing into pulmonary artery.

Accuracy of echocardiography in diagnosing total anomalous pulmonary venous return.

OBJECTIVE: Total anomalous pulmonary venous return is an uncommon cyanotic congenital heart defect. Echocardiography is the initial diagnostic tool. Complimentary non-invasive modalities like cardiac computerized tomographic angiography and cardiac magnetic resonance imaging have replaced the need for cardiac catheterization in difficult cases. This study aimed to determine the accuracy of echocardiography in diagnosing total anomalous pulmonary venous return, and to determine the factors that may decrease its sensitivity. METHODS: This was a cross-sectional study conducted at the Aga Khan University Hospital Karachi, Pakistan from January 2010 to August 2016. All patients who were diagnosed with Total anomalous pulmonary venous return on echocardiography and had subsequent confirmation either on cardiac CT angiography or surgery were included. The diagnostic accuracy of echocardiography was expressed as sensitivity. Previously described taxonomy was used to define diagnostic error. Univariate and multivariate analysis were done by logistic regression OR (95% CI) were reported to identify factors causing the diagnostic error. RESULTS: High diagnostic sensitivity (81%) was found in isolated total anomalous pulmonary venous return and low (27%) in heterotaxy and mixed (20%) varieties. Poor acoustic windows and right isomerism were found to be significant factors responsible for the diagnostic error on multivariate analysis. CONCLUSION: Echocardiography can diagnose isolated total anomalous pulmonary venous return with high accuracy. Use of additional modalities may be required for a complete diagnosis in cases with mixed variety, heterotaxy and poor acoustic windows.

Is Grown Up Congenital Heart (GUCH) disease different in a developing country?

In the current era grown up congenital heart disease (GUCH) patients undergoing surgical interventions are increasing. Most of the interventions in the developed countries are either complex or redo-operations in patients who had previously undergone repair, palliation or correction. However, in the developing countries most of the interventions are primary and corrective. This descriptive retrospective study comprised GUCH patients who underwent surgical intervention for congenital heart disease (CHD) at Aga Khan University Hospital, Karachi, from January 2006 to December 2015. A total of 195 patients had been treated surgically with a mean age of 31.0±13.5 years. Majority of the patients underwent surgical interventions for closure of atrial 109(55.3%) and ventricular 51(26.2%) septal defect. The most common complications were prolonged ventilation 16(8.1%). Overall mortality was 4(2.1%). GUCH in our practice is for primary procedure with simple diagnosis that should have been treated before reaching adulthood as is done in the developed countries.

Conversion of cardiac bypass into an extracorporeal membrane oxygenation circuit: a case from Pakistan.

A 35 days old neonate with d-loop transposition of great arteries, underwent an arterial switch operation following which he developed hypotension attributed to left ventricular failure. During cardiopulmonary resuscitation decision was made to place him on cardiac bypass again. Due to limited resources and unavailability of a specialized extracorporeal membrane oxygenator machine, the CPB was modified and converted an ECMO. The neonate was successfully decannulated after 72 hours and discharged home after 3 weeks of the operation without any sequel. ECMO is a viable option in developing countries and may help in improving the outcome especially in neonatal congenital heart disease.

Hospital Resources and Practice Patterns for Congenital Cardiac Surgery in Pakistan: A Nationwide Status Report.

Objective: Low- and middle-income countries such as Pakistan bear the brunt of the global burden of congenital heart disease (CHD), This nationwide study assessed hospital resources and surgical practice patterns pertaining to CHD surgery across Pakistan. Methods: A nationwide hospital facility survey was conducted, targeting all health centers performing CHD surgery in Pakistan. Descriptive data analysis was performed, with categorical variables being presented as frequencies and percentages and continuous variables being reported as means and standard deviations. Results: Seventeen surgeons across 17 different centers participated in the study. Eight of 17 (47%) surgeons were under 45 years of age, while the rest were older than 45 years. More respondents were employed at public/government-owned hospitals (11/17, 65%) and at specialized cardiac centers (13/17, 76%), with a majority of centers reported as having a special ICU designated for congenital cardiac patients (11/17, 65%). With regard to operative volume, 7 of 17 (41%) centers reported more than 350 cases per year, with 7 of 17 reported >10 neonatal cases per year (6/12, 50%). Only one center reported national and international collaborations for research, most centers carrying out less than five research projects in a year. Conclusion: This study has identified key areas that require the attention of local administrative bodies as well as international organizations and societies. The existing centers in Pakistan are unable to meet the demands of the population, leaving a large number of untreated patients, especially for neonatal lesions.

Transatrial access for left atrial pressure (LAP) monitoring line placement in arterial switch operation (ASO) in neonates.

Left Atrial pressure monitoring is a useful and accurate method to guide Left ventricle filling in the patients who undergo Arterial switch operation for transposition of great arteries. We have used a different technique in three TGA patients for LA pressure monitoring line placement. After cleaning and draping,right internal jugular vein (rt IJV) located through 22G venous cannula, guide wire was put in followed by sliding the 22G x 8cm vygon arterial catheter over the guide wire into the right atrium that was directed transatrially into LA by the operating surgeon during atrial septum repair. The catheter was secured by silk on the neck and dressed with transparent dressing and was kept for a period of 48-72 hrs. LA pressure monitoring is helpful in anticipating LV dysfunction in ASO.

Midterm results of bovine jugular vein conduit for right ventricular outflow tract reconstruction.

OBJECTIVE: To evaluate the midterm results of Contegra conduit. METHODS: The retrospective study comprised patient record at Aga Khan University Hospital, Karachi, of conduits implanted between May 2007 and June 2012. Data collection was made from the clinical notes and from serial echocardiograms by a single cardiologist. The last followup echocardiography was done at the time of data collection in June 2012. SPSS 19 was used for statistical analysis. RESULTS: A total of 18 conduits had been implanted (16-22 mm) during the study period. Median age at the time of surgery was 9 years (range: 2.5-16 years). Early mortality was seen in 3 (16.66%) patients, but none was Contegra related. Of the remaining 15 patients, 2 (13.33%) with a diagnosis of Pulmonary Atresia-Ventricular Septal Defect with hypoplastic peripheral Pulmonary Arteries (PA), developed severe distal pressure gradient (50 mmHg) across Contegra over a median period of 18 months (range: 12-24 months), with resultant severe regurgitation and needed percutaneous intervention. There was no thrombosis, calcification, anuerysmal dilation or late deaths. CONCLUSION: At midterm followup, Contegra conduit was associated with low re-intervention rates with satisfactory haemodynamic results. However, long-term durability must be determined for this conduit, especially in patients with Pulmonary Atresia-Ventricular Septal Defect with hypoplastic peripheral Pulmonary Arteries.

Oesophageal duplication cyst presenting as haemoptysis.

Duplications of the alimentary tract include a variety of cysts, diverticula, and tubular malformations, all believed to have embryological origin. The cysts are most commonly found in children, and the diagnosis is made in infancy in the majority of patients. We report a case of a two-and-a-half year old child, presenting with the history of repeated episodes of haematemesis. Upper GI endoscopy was unremarkable and the chest x-ray showed no pathology. Computed tomography (CT) angiogram revealed soft tissue density lesion in the right chest at the level of T6. Right thoracotomy suggested a cystic mass close to the oesophagus which was shown on histopathology to be lined with gastric mucosa consistent with oesophageal duplication cyst. To the best of our knowledge, this is the first case of its kind reported from Pakistan.

Lethal Fungal Aortitis In Surgically Corrected Supravalvular Aortic Stenosis In A Child With Williams Syndrome.

Williams syndrome (WS), is a multisystem disorder occurring in 1 in 10,000 live births with supravalvular aortic stenosis (SVAS) being the most common cardiovascular manifestation. We present the case of a 2.5 years old male, a known case of WS who presented with cognitive delay, a history of right-sided stroke and left hemiplegia. Echocardiography revealed severe SVAS with a gradient of 105 mmHg. The diameter of the Sino tubular junction was 4 mm. Computerized tomography angiogram showed diffuse stenosis of ascending aorta with intraluminal thrombus. At surgery, the ascending aorta was augmented with autologous pericardial patches and end-to-end anastomosis of the proximal and distal aorta completed the reconstruction. The patient was discharged in a stable condition. He presented 6 weeks post-op with a pulsating pseudoaneurysm through the sternal wound. Emergency surgery with the removal of fungal vegetation and reconstruction of the ascending aorta was performed. He expired due to fungal sepsis a week later.

Factors associated with poor outcomes after congenital heart surgery in low-resource setting in Pakistan: insight from the IQIC Registry - a descriptive analysis.

OBJECTIVE: This study aimed to assess the International Quality Improvement Collaborative single-site data from a developing country to identify trends in outcomes and factors associated with poor outcomes. DESIGN: Retrospective descriptive study. SETTING: The National Institute of Cardiovascular Diseases, Karachi, Pakistan. PARTICIPANTS: Patients undergoing surgery for congenital heart disease (CHD). OUTCOME MEASURE: Key factors were examined, including preoperative, procedural and demographic data, as well as surgical complications and outcomes. We identified risk factors for mortality, bacterial sepsis and 30-day mortality using multivariable logistic regression. RESULTS: A total of 3367 CHD surgical cases were evaluated; of these, 59.4% (2001) were male and 82.8% (2787) were between the ages of 1 and 17 years. Only 0.2% (n=6) were infants (≤30 days) and 2.3% (n=77) were adults (≥18 years). The in-hospital mortality rate was 6.7% (n=224), and 4.4% (n=147) and 0.8% (n=27) had bacterial sepsis and surgical site infections, respectively. The 30-day status was known for 90.8% (n=3058) of the patients, of whom 91.6% (n=2800) were alive. On multivariable analysis, the adjusted OR for in-hospital mortality was 0.40 (0.29-0.56) for teenagers compared with infancy/childhood and 1.95 (1.45-2.61) for patients with oxygen saturation <85%. Compared with Risk Adjustment for Congenital Heart Surgery (RACHS-1) risk category 1, the adjusted OR for in-hospital mortality was 1.78 (1.1-2.87) for RACHS-1 risk category 3 and 2.92 (1.03-8.31) for categories 4-6. The adjusted OR for 30-day mortality was 0.40 (0.30-0.55) for teenagers and 1.52 (1.16-1.98) for patients with oxygen saturation <85%. The 30-day mortality rate was significantly higher in RACHS-1 risk category 3 compared with category 1, with an adjusted OR of 1.64 (1.06-2.55). CONCLUSIONS: We observed a high prevalence of postoperative infections and mortality, especially for high-risk procedures, according to RACHS-1 risk category, in infancy/childhood, in children with genetic syndrome or those with low oxygen saturation (<85%).

Recommendations for developing effective and safe paediatric and congenital heart disease services in low-income and middle-income countries: a public health framework.

The global burden of paediatric and congenital heart disease (PCHD) is substantial. We propose a novel public health framework with recommendations for developing effective and safe PCHD services in low-income and middle-income countries (LMICs). This framework was created by the Global Initiative for Children's Surgery Cardiac Surgery working group in collaboration with a group of international rexperts in providing paediatric and congenital cardiac care to patients with CHD and rheumatic heart disease (RHD) in LMICs. Effective and safe PCHD care is inaccessible to many, and there is no consensus on the best approaches to provide meaningful access in resource-limited settings, where it is often needed the most. Considering the high inequity in access to care for CHD and RHD, we aimed to create an actionable framework for health practitioners, policy makers and patients that supports treatment and prevention. It was formulated based on rigorous evaluation of available guidelines and standards of care and builds on a consensus process about the competencies needed at each step of the care continuum. We recommend a tier-based framework for PCHD care integrated within existing health systems. Each level of care is expected to meet minimum benchmarks and ensure high-quality and family centred care. We propose that cardiac surgery capabilities should only be developed at the more advanced levels on hospitals that have an established foundation of cardiology and cardiac surgery services, including screening, diagnostics, inpatient and outpatient care, postoperative care and cardiac catheterisation. This approach requires a quality control system and close collaboration between the different levels of care to facilitate the journey and care of every child with heart disease. This effort was designed to guide readers and leaders in taking action, strengthening capacity, evaluating impact, advancing policy and engaging in partnerships to guide facilities providing PCHD care in LMICs.

A Stab In The Forbidden Territory.

Penetrating heart injuries are associated with higher mortality rates. Coronary lesions caused by penetrating trauma are considered even rarer and universally fatal. We present a case of a fortunate survivor who had complete transection of left anterior descending (LAD) artery with right ventricular (RV) tear after being stabbed by knife, arriving in emergency unit with massively bleeding chest wound. Complex cardiac trauma involving coronaries and cardiac chambers is a challenge to surgeons if patients miraculously reach the hospital alive. This patient had complete transection of LAD artery with penetration into RV cavity, he was successfully managed by timely and prompt surgical intervention by on call team. This case highlights the importance of team dynamics working in harmony during emergency situations, we stress upon conducting routine drills to train surgical residents, perfusionists and operation theatre staff.

Case report: Pulmonary artery thrombosis in cyanotic congenital heart disease.

Cyanotic congenital cardiac anomalies increase the risk of thrombosis. In pediatric congenital heart disease, the diagnosis of pulmonary artery thrombosis is rare. We reported an 11-year-old male child who developed progressive shortness of breath over six months. He was subsequently referred to the National Institute of Cardiovascular Diseases, Karachi, Pakistan. The child was diagnosed as a case of double outlet left ventricle, levo-transposition of the great arteries, pulmonary stenosis, ventricular septal defect and thrombus in the main pulmonary artery with extension into the branch pulmonary arteries. He underwent thromboendarterectomy with a Glenn shunt and made an uneventful postoperative recovery.

Surgical rescue of embolized amplatzer devices.

BACKGROUND AND AIM: Transcatheter closure of atrial septal defect (ASD) and patent ductus arteriosus (PDA) with Amplatzer septal/duct occluder (ASO/ADO) is an established, safe, and efficient procedure with high success. However, device embolization remains a major complication requiring immediate intervention (either percutaneous or surgical) for retrieval and correction of the heart defect. The aim of this study is to share the experience of managing embolized ASO/ADO. METHODS: Of the 284 cases of device closure performed from October 2002 to December 2010, four patients (1.4%) had device embolization requiring immediate surgical retrieval. Two adult female patients with secundum ASD had ASO device implanted. One embolized to the right ventricle and the other into the ascending aorta. An eight-month-old boy and a four-year-old girl with hypertensive PDA had device closure. Device embolization occurred into the descending aorta and right pulmonary artery, respectively. RESULTS: All four devices were retrieved and the defects closed successfully with a low morbidity and no mortality. CONCLUSION: Careful consideration should be given to surgical or transcatheter closure of a heart defect. Life-threatening complications although rare can occur. Our experience strongly suggests that these devices should only be inserted in facilities where cardiac surgical support is immediately available.