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%).

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.