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.

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%).

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.

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.

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.

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.

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.

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.

The Glenn procedure: Clinical outcomes in patients with congenital heart disease in pakistan.

Objectives: Congenital heart defects (CHDs) affect more than 40,000 children annually in Pakistan. Approximately 80' of patients require at least one surgical intervention to achieve a complete or palliative cardiac repair. The Glenn shunt, a palliative procedure is established between superior vena cava (SVC) and the right pulmonary artery to provide an anastomosis offering minimal risk to patients with univentricular heart disease. The aim of this study was to assess the clinical outcomes of the Glenn shunt procedure in patients with complex congenital heart diseases in a developing country like Pakistan. Materials and Methods: A retrospective chart review was conducted on patients who underwent a bidirectional Glenn shunt procedure from July 2006 to June 2017. Data were collected on a structured questionnaire and analyses performed on SPSS version 22. Frequencies and percentages were computed for categorical variables while mean and standard deviation for continuous variables where appropriate. Results: A total of 79 patients underwent the Glenn shunt procedures. The median age was 1.9 years and 54.5' were male. Tricuspid atresia was the primary diagnosis in 30.4' of the patients. Common morbidities included arrhythmias (6.3'), pleural effusion (8.9'), wound infection (3.8'), pneumonia (2.5'), and seizures (3.8'); reopening was required in 2.5' of the patients and 8.8' were readmitted within 30 days of index operation. There were three (3.8') deaths in total. Conclusions: Bidirectional Glenn shunt procedure can be performed safely in patients with ideal characteristics as the first stage palliation and has favorable results with acceptable rate of complications.

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.

Right ventricular outflow reconstruction with handmade valve conduit - A short experience from a developing country. Case series.

OBJECTIVES: Right ventricular outflow tract continuity abnormalities are one of the most commonly encountered entities in the field of congenital cardiac surgery. Various strategies including homograft, valve conduit, Contegra are used to restore continuity between right ventricle and pulmonary artery. In countries like Pakistan these may not be easily available and affordable. We report the experience of our short observational study of using a handmade trileaflet valve conduit to reconstruct the right ventricular outflow tract. METHODOLOGY: From September 2015 to December 2016, a total of 15 patients with different congenital heart diseases underwent open-heart surgery at our institute. Restoration of right ventricular to pulmonary artery continuity was achieved using handmade valve conduit utilizing bovine pericardium and thin sheet PTFE sheets (0.1 mm) as conduit and valve respectively. RESULTS: Patients ranged from 1 to 16 years. Seven patients had previous palliation including 4 blalock taussig (BT) Shunts and 3 pulmonary artery (PA) banding. Postoperative complications were observed in 4 patient including 2 in hospital deaths and 2 required interventions. One patient developed aneurysm at RV- conduit junction requiring surgical repair and the other underwent conduit dilatation for moderate to severe stenosis (gradient 60 mmHg). No significant regurgitation was observed in this series. Overall postoperative gradients were stable with mean gradient 25.3 mmHg (8 mmhg - 60 mmHg). CONCLUSION: The use of handmade valve conduits has acceptable morbidity and mortality. These are cost effective alternatives in this part of the world, where well-established conduits have cost implications and uncertain availability.

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.

Conversion from Off to On-Pump Coronary Artery Bypass Grafting. Is it Avoidable?

Background With the emergence of new technologies to stabilize the heart off-pump coronary artery bypass grafting (OPCAB), there is an increasing trend that is being observed throughout the world. In certain circumstances, OPCAB needs to be converted to on-pump CABG (ONCAB). In this study, we aim to identify certain risk factors mandating conversions and their associated short-term outcomes. Methods After approval from the institutional ethical review committee and exemption from informed consent, retrospective data of 100 patients meeting the inclusion criteria who underwent OPCAB operations at our institution from August 2018 to July 2019 were included. Preoperative, intraoperative, and postoperative variables were recorded and compared in conversion and non-conversion groups. This study was conducted at the National Institute of Cardiovascular Diseases, Karachi, Pakistan. Results A total of 100 patients were included in this study, out of which 82% (82) were male, with age ranging between 18 and 77 years with a mean age of 56.34 ± 8.3 years. In nine of the cases, OPCAB was emergently converted to ONCAB due to arrhythmias. In nine (9%) cases, off-pump CABG was emergently converted to on-pump CABG (ONCAB). Emergent conversion was due to arrhythmias in five cases, due to hypotension during OM graft in two cases, and due to hypotension during ramus graft for the remaining two cases. The emergent conversion was significantly associated with higher New York Heart Association (NYHA) functional classification and comorbid conditions such as chronic obstructive pulmonary disease (COPD). Conclusion Emergency conversion from off-pump to OPCAB is the most catastrophic event causing higher morbidity and mortality. Conversion rate was observed to be 9% with arrhythmias being the common cause and patients with higher NYHA status and COPD at baseline were found to be at increased risk of emergency conversion. Considering our results in patients with diagnosed COPD and higher NYHA status, the decision for off-pump CABG should be wisely taken carefully weighing the risks and benefits.

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.

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.

Anomalous Left Coronary Artery from Pulmonary Artery: An Important Cause of Ischemic Mitral Regurgitation in Children.

INTRODUCTION:  Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly. The usual presentation in infancy is inconsolable crying or congestive cardiac failure, both due to myocardial ischemia. Survivors after infancy have improved left ventricular function but continue to have mitral regurgitation due to papillary muscle ischemia. The present study emphasizes the importance of unexplained mitral regurgitation as a clue to the diagnosis. PATIENTS AND METHODS:  Patients with the diagnosis of ALCAPA operated between June 2017 and May 2018 were enrolled. Their ages at diagnosis, electrocardiography, and echocardiography findings were noted. A selective angiogram of the right coronary artery was done in all. Results of surgical reimplantation were analyzed. Postoperative data were collected, including ventricular function and mitral regurgitation. RESULTS:  Six patients were included. Clinical signs of cardiac failure were present in two patients, and a systolic murmur was heard in all. The mean left ventricular ejection fraction was 52 ± 12%. Mitral regurgitation was present in all of the patients. The right coronary artery was dilated (Z score > 2.5) in all except one. Selective right coronary angiogram and cardiac computerized tomography angiogram (CTA) were performed in all. Coronary reimplantation was successfully done. Follow-up echocardiography showed improved left ventricular ejection fraction and degree of mitral regurgitation in all patients. CONCLUSION:  ALCAPA is an uncommon congenital anomaly, the diagnosis of which can be missed, particularly in late presenters. Unexplained mitral regurgitation should always raise the suspicion of this anomaly. Surgical intervention has excellent results with an improvement of left ventricular function and mitral regurgitation.

Technical Varieties of Ross Procedure: A Case Series.

Replacement of aortic valve with the pulmonary autograft is carried out through the Ross procedure due to its potential for growth, durability in pediatric population, and absence of anticoagulation. This case series reports the postoperative outcome of two technical variations of Ross procedure in eight patients who underwent surgery from January 2007 to December 2016. The dominant valvular hemodynamic indication was aortic regurgitation. The techniques employed for Ross procedure included free standing root replacement in six patients and modified root replacement with autograft stabilisation using Dacron interposition graft in two patients. Right ventricular outflow tract (RVOT) conduit reconstruction was achieved by utilisation of Contegra bio prosthesis in four patients; and use of manually constructed valved conduit comprising of PTFE (polytetrafluoroethelene) membrane and bovine pericardial tube in remaining four patients. There was no mortality and no re-intervention. The technical varieties of Ross procedure offer freedom from need of anti-coagulation, mortality, and RVOT conduit failure in younger adults.

Malignant Pheochromocytoma with Widespread Bony and Pulmonary Metastases.

Pheochromocytoma is a rare benign tumor of the adrenal gland. A select few cases may be malignant, and metastatic cases are exceedingly rare. It often presents with symptoms of catecholamine excess, such as sweating, palpitations, headaches, and characteristic paroxysmal hypertension. Due to its diffuse symptoms, it is difficult to diagnose and is often diagnosed late. We describe the unique case of a 44-year-old female patient who presented with uncontrolled hypertension and vomiting, accompanied by lower back pain. She was diagnosed with malignant pheochromocytoma with multiple metastases to the lungs, vertebrae, scapulae, and skull. Because of the advanced state of her disease, the patient was started on treatment with the chemotherapeutic combination of cyclophosphamide, vincristine, and dacarbazine. However, she had a complicated hospital course and died because of aspiration pneumonia and sepsis.

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.

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.