Massive left pulmonary artery aneurysm with a co-existing patent ductus arteriosus in a five-year-old female child: A case report.

Pulmonary Artery Aneur ysm (PAA), whether congenital or acquired, is a rare diagnostic find ing com pare d to aor tic aneur ysms. There have been fe w cases where PA As were documented as a complication of untreated Patent Ductus Ar teriosus (PDA) due to long-standing Pulmonary Arterial H ypertension (PAH). However, it is quite rare for a case of PAA to be reported with co-existing PDA without PAH. This report highlights a case of a five -year-old girl who was presented with palpitations, easy fatigability, fever, c yanos is, and vomiting. A Chest X-ray s howed mo derate cardiomega ly. A PDA of 6 mm was diagnosed on Transthoracic E chocardiog rap hy ( TTE ) and a large cavity con necte d with LPA raised suspicion of a possible LPA aneur ysm. A Chest CT scan confirm ed the diagnosis of a saccular aneurysm, originating from the distal part of the main Left Pulmonary Artery (LPA) just proximal to the point of bifurcation into lobar branches, measuring 7.5x6.5 cm. During surgery, the aneurysm was opened, emptied with suction and closed without resecting the aneur ysmal walls. The patient had an uneventful post-op course and is doing well during regular interval follow up visits.

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%).

Accidental Penetrating Thoracic Injury with a Screwdriver in a Child: A Case Report.

Accidental penetrating injuries are common among children, either with a sharp or a blunt object. The screwdriver is an uncommon weapon; therefore, injuries caused by it represent an even rarer subset. Inadvertent injuries in the chest with a screwdriver as a stabbing weapon are extremely unusual. Penetrating chest injuries can be fatal if they cause wounds in the cardiac chambers or major thoracic vessels. We describe a 9-year-old child with an unintentional penetrating thoracic injury caused by a screwdriver. An explorative left anterior thoracotomy showed that the tip of the implanted screwdriver was lying near the left subclavian vessels and the apex of the lung, but it did not perforate any of those. The screwdriver was dislodged, and the wound was closed. The patient had an event-free 1-week hospital stay.

The Spectrum of Pediatric Cardiac Procedures and Their Outcomes: A Six-month Report from the Largest Cardiac Facility in Sindh, Pakistan.

Introduction Congenital heart disease (CHD) is the most common birth defect globally, with low-to-middle income Asian countries registering the highest incidence. Every year, 60,000 babies are born with varying severity of CHD in Pakistan. But the country has only three pediatric intensive care units (PICU) fully dedicated to child cardiac surgery patients. The focus of this study is to analyze the spectrum of pediatric cardiac surgical procedures performed for the management of CHD and their outcomes in a cardiac PICU in Pakistan. Methods In this analysis, all surgical records of children admitted to the PICU of National Institute of Cardiovascular Diseases (NICVD), Karachi, from October 2018 to March 2019 were included. It is a 14-bed, state-of-the-art cardiac PICU, which provides high-quality care to critical post-surgical patients. Results The surgical records of 537 patients were extracted for the purpose of our study, which accounted for 89.5 of post-operative patients admitted in the PICU per month and three per day. Tetralogy of Fallot (TOF) was the most commonly treated anomaly (n = 161; 29.9%) in the facility, followed by ventricular septal defect (n = 107; 19.9%). The overall mortality rate was 5.4% (n = 29), out of which 27.5% (n = 8) were TOF-related. Conclusions There is a very high burden of patients on the cardiac PICUs in low-to-middle income Asian countries. Despite the lack of resources, the high- quality care provided by pediatric cardiac critical-care specialists at these PICUs has ensured favorable outcomes and a mortality rate as low as that in any of the developed countries.

Incidence of Aortic Regurgitation in Association with Type of Ventricular Septal Defects and its Immediate and Intermediate Outcome after Surgical Closure.

Introduction Ventricular septal defect (VSD) is one of the more common congenital heart defects, and aortic regurgitation (AR) is its major complication if it remains unrepaired. We aim to determine the AR incidence in various types of VSD, its immediate and intermediate six to 12-month post-VSD repair outcomes of AR. Methods We conducted a retrospective review of medical records of all children aged 18 years or younger who were diagnosed with single VSD at our institution from 2016 to 2018. VSD was classified according to its location and relation to the tricuspid annulus and semilunar valve. AR severity grading was done according to the American Society of Echocardiography, and vena contracta width (VC) was taken as the main parameter for severity. We defined trivial-to-mild AR as VC width less than 0.3 cm, moderate AR was 0.3-0.6 cm VC width, and severe AR was VC width of more than 0.6 cm. Immediate and intermediate outcomes of surgical closure, such as residual VSD and AR, were observed. Results One hundred ninety patients with isolated single VSD were included in the study. Of those, 114 patients had perimembranous VSD (60.0%), 64 patients had muscular VSD (33.7%), and 12 patients had supracristal VSD (6.3%). The median age of our study cohort was six months, with a male to female ratio of 1.3:1. Aortic valve prolapse (28.9%; n = 55) and AR (23.2%; n = 44) were the most common findings on echocardiographic evaluation of VSD patients. Most cases of VSD with AR had trivial-to-mild AR, (68.2%; n = 30). AR was most commonly seen in supracristal VSD (83.3%; n = 10) followed by perimembranous VSD (28.9%; n = 33). VSD closed spontaneously in 34 patients (17.9%) and 98 patients (51.6%) patients underwent surgery. Residual VSD after surgical closure was present in 57.1% (56) and 17.3% (17) of the patients immediate postoperatively and six- to 12-month postoperative follow-up, respectively. Similarly, residual AR after surgical closure of VSD was present in 32.7% (32) and 15.3% (15) of the patients immediate postoperatively and six- to 12-month postoperative follow-up, respectively. Conclusion The incidence of AR with VSD was very high in our study; AR was most commonly associated with supracristal VSD. After surgical repair, mild AR decreased with time. Early corrective surgery of VSD can prevent this complication and help improve outcomes.

Senning Procedure for Transposition of the Great Arteries in a Patient with Situs Inversus Totalis and Dextrocardia.

The arterial switch operation is considered as standard therapy for isolated D-transposition of the great arteries. However, in under-developed countries, patients still present with D-transposition of the great arteries beyond the neonatal age redering them unsuitable for anatomical repair. These children are often offered physiological repair, i.e. Senning or Mustard procedure. We describe our experience of a successful Senning procredure in a 4.6-year girl, who had atrial and visceral situs inversus totalis, dextrocardia and d-transposition of the great arteries. To our knowledge, this is the first reported case of this operation in a patient with such unique anatomy.

Total Cavopulmonary Connection for Functionally Single Ventricle without Cardiopulmonary Bypass Support.

A heart with two atriums but one ventricle, an anatomy with a unique physiology, is responsible for many creative surgical and interventional approaches in history. Different surgical techniques have been used to address this strange physiology of parallel circulation. All these attempts met with failure till the concept of Fontan circulation was described. Currently, controversy exists between multistage vs. single stage total cavopulmonary connections. Total cavopulmonary connection is the only definitive procedure performed to provide palliation for patients with complex congenital heart defects which cannot support a biventricular circulation. We report a case with tricuspid atresia with transposition of great arteries and pulmonary stenosis with persistant left-sided superior vena cava and functionally single ventricle. Patient successfully underwent single stage extra-cardiac total cavopulmonary connection. In this case, bilateral Glenn with extra-cardiac inferior vena cava to main pulmonary artery shunt was performed off-pump.