A rare case of large true main pulmonary artery aneurysm managed surgically: A case report.

INTRODUCTION & IMPORTANCE: Pulmonary Artery Aneurysm is defined as the localized dilation of the pulmonary artery >1.5 times the upper normal limit or pulmonary artery measuring 4 cm. Pulmonary artery aneurysm is considered as a rare disorder having an incidence of 1 in 14,000 post-mortem examinations. CASE PRESENTATION: Presented below is a case of a 28 year old gentleman, who presented with exertional dyspnea and orthopnea and was diagnosed with having a pulmonary artery aneurysm of 76 cm × 56 cm × 53 cm arising from the main pulmonary artery upon Computed Tomography Scan which is a rare finding according to the available literature. CLINICAL DISCUSSION: Clinical manifestations of pulmonary artery aneurysm are varying and rarely occur. However the use of radiological imaging has aided in the diagnosis. No specific treatment guidelines have been mentioned yet in the literature however, medical management, surgical resection and endovascular therapy are one of the multiple options available. CONCLUSION: Pulmonary Artery Aneurysm presents with non-specific symptoms which makes the diagnosis very challenging for the physicians, in process, delaying the accurate management of the disease. However, pulmonary artery aneurysm must be considered as a differential diagnosis and appropriate management options, whether medical or surgical should be opted for keeping in mind the size and the complications of the disease.

Mediastinal seminoma in a pediatric patient managed with multi-modality treatment approach: A case report.

INTRODUCTION: Mediastinal seminomas arise due to abnormal migration of the totipotent stem cells during development. They occur very occasionally and makes up about 0.5-5 % of all mediastinal tumors. These usually occur in middle aged men, between 20 and 40 years. In pediatric age group, it is considered as an extremely rare finding, accounting for 3 %-7 % of all primary germ cell tumors. CASE PRESENTATION: We report a rare case of a 16-year-old boy with biopsy proven seminomatous type of germ cell tumor in anterior mediastinum who presented with productive cough and progressive dyspnea. The occurrence of mediastinal seminoma especially in pediatric population is a very infrequent finding, hence, very little literature is found in this regard as per our knowledge. DISCUSSION: The incidence of primary mediastinal germ cell tumors is approximately around 3 %-6 % of all the germ cell tumors in pediatric population. Diagnosis of mediastinal seminomas need a multipronged approach and treatment is associated with multidisciplinary procedures including chemotherapy combined with subsequent surgical resection for long term survival benefits. CONCLUSION: From the above presented case, it can be safely concluded that multimodality treatment approach of chemotherapy combined with surgical resection could ensure long-term survival benefits in extra-gonadal mediastinal seminomas.

Risk factors and outcomes of prolonged cardiopulmonary bypass time in surgery for adult congenital heart disease: a single-center study from a low-middle-income country.

BACKGROUND: Prolonged cardiopulmonary bypass time (prolonged CPBT; PCPBT) during operations for adult congenital heart disease (ACHD) may lead to worse postoperative outcomes, which could add a significant burden to hospitals in developing countries. This study aimed to identify risk factors and outcomes of PCPBT in patients undergoing operations for ACHD. METHODS: This retrospective study included all adult patients (≥18 years) who underwent cardiac surgery with cardiopulmonary bypass for their congenital heart defect from 2011-2016 at a tertiary-care private hospital in Pakistan. Prolonged CPBT was defined as CPBT>120 minutes (65th percentile). RESULTS: This study included 166 patients (53.6% males) with a mean age of 32.05±12.11 years. Comorbid disease was present in 59.0% of patients. Most patients underwent atrial septal defect repair (42.2%). A total of 58 (34.9%) of patients had a PCPBT. Postoperative complications occurred in 38.6% of patients. Multivariable analysis adjusted for age, gender and RACHS-1 Categories showed that mild preoperative left ventricular (LV) dysfunction was associated with PCPBT (OR=3.137 [95% CI: 1.003-9.818]), while obesity was found to be protective (0.346 [0.130-0.923]). PCPBT was also associated with a longer duration of ventilation (1.298 [1.005-1.676]), longer cardiac ICU stay (1.204 [1.061-1.367]) and longer hospital stay (1.120 [1.005-1.247]). CONCLUSIONS: While mild preoperative LV dysfunction was associated with PCPBT, obesity was found to be protective. Postoperatively, PCPBT was associated with longer duration of ventilation, cardiac ICU stay, and hospital stay. Operations with shorter CPBT may help minimize the occurrence and impact of these postoperative adverse outcomes especially in resource-constrained developing countries.

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.

Anomalous origin of the left coronary artery from the pulmonary artery: A surgical certainty.

This study aims to assess the clinical presentation and the outcomes of a surgical correction of an anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). A retrospective review was carried out on the charts of six patients admitted for ALCAPA surgery at Aga Khan University Hospital, Karachi from March 2017 to May 2018.Dyspnoea, palpitation, poor feeding, fatiguability, pallor and a murmur of mitral regurgitation were the main presenting features. The pre-operative median left ventricular ejection fraction (LVEF) was 64%. Coronary reimplantation was performed in all the patients with a mitral valve repair being done in only one patient. The mea n LV EF was 66 .3%,p ost- ope rativel y. Mitra l regurgitation (MR) improved in patients post-operatively with trace in 2 patients and mild MR in one. Surgical correction by coronary re-implantation yields favourable outcomes in ALCAPA and significantly reduces the morbidity and mortality rates associated with the disease.

Aberrant right subclavian artery with atrial septal defect: Simultaneous repair via median sternotomy.

INTRODUCTION: Aberrant right subclavian artery, also known as arteria lusoria, is one of the rarer congenital vascular malformations of the left sided aortic arch which arises distal to the left subclavian artery, following a retroesophageal course to the right side causing compression of the trachea or esophagus and resulting in a variety of symptoms most commonly dysphagia. PRESENTATION OF CASE: We report a case of a two and a half year old child who was diagnosed as having as having an aberrant right subclavian artery with an ostium secundum atrial septal defect and underwent simultaneous repair of both conditions via a median sternotomy. DISCUSSION: Aberrant right subclavian artery (ARSA) has a prevalence of 1.8 % and most commonly presents in fourth or fifth decade of life. Various surgical approaches have been described to repair this anomaly over the past years, each with its own limitations and complications. However, the median sternotomy approach provides adequate exposure during division and re-implantation of the vessel and simultaneously allows repair of any intra-cardiac defect. CONCLUSION: We believe that the best exposure for the correction of aberrant right subclavian artery is via a median sternotomy, especially in pediatric patients associated with other cardiac anomalies.

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.

Short And Midterm Outcome Of Fallot's Tetralogy Repair In Infancy: A Single Center Experience In A Developing Country.

BACKGROUND: Primary repair of ToF between 3-12 months is the preferred mode of treatment worldwide, with low surgical mortality. This study reviews our experience of ToF repair in infancy and its short and midterm outcomes in a single centre from a developing country. METHODS: Data of all patients with Tetralogy of Fallot repair during infancy from January 2007 to Feb 2018 was reviewed. Preoperative, operative, and postoperative data was analysed. Outcome of the infants was assessed through discharge/death, low cardiac output syndrome (LCOS), prolonged intubation, duration of cardiac intensive care unit (CICU) and hospital stay. RESULTS: Forty-four patients who underwent TOF repair in infancy during this period were included. The mean age and weight were 9.39±2.32 and 7.20±1.30 respectively, 77.3% (34 patients) were male, 68.18% (30 patients) had saturation >75%. Mean intubation period was 4.05±6.58 days, 12 (27.3%) patients developed LCOS, mean cardiopulmonary bypass (CPB) time, aortic cross clamp (ACC) time and ionotropic score were 133.52±62.4, 98.66±58.62 and 33.27±71.13 respectively. Mean CICU and hospital stay was 6.60±7.18 and 12.05±7.74 respectively. Five (11.3%) patients expired in postoperative period. Baseline saturation ≤75% is independent risk factor for LCOS and prolong intubation period. In the last six years our mortality decreased to 8% from 15.7% during the previous six years, while our mean intubation duration, CPB time, ACC, hospital stay and CICU stay have all shown improvement. CONCLUSIONS: TOF repair during infancy is safe procedure in expert hands with acceptable morbidity and mortality. Baseline saturation ≤75% is independent risk factor for LCOS and prolonged intubation period. Last six years have shown considerable improvement in our surgical morbidity and mortality due to improvement in surgical expertise.

Herceptin responsive lung adenocarcinoma in the setting of bilateral synchronous lung primaries and breast carcinoma.

The incidence of human epidermal growth factor receptor 2 (Her-2) mutations in lung adenocarcinoma is approximately 3%; however, its significance in the management of these lung cancers remains under investigation. We describe an incidental but unique opportunity to evaluate the response to treatment with herceptin in a patient with bilateral synchronous lung primaries in conjunction with breast carcinoma. Interval imaging following surgical resection of the squamous cell carcinoma while on herceptin treatment delineated the radiological regression of the Her-2 positive lung adenocarcinoma. We feel that this case highlights the potential role for herceptin treatment in Her-2 positive lung adenocarcinomas and demonstrates the importance of screening for these mutations.