Circumflex aortic arch: presentation across various age groups: a case series.

Circumflex aortic arch (CAA) is a rare congenital anomaly where the aortic arch crosses the midline, posterior to the esophagus and trachea, and descends on the contralateral side. If patent ductus arteriosus (PDA) is present, this forms a true vascular ring. CAA can compress the trachea and esophagus leading to a myriad of symptoms which can present at any age. We describe our experience with three patients of the CAA, presenting across different age groups.

Spontaneous pneumomediastinum and subcutaneous emphysema in patients with COVID-19.

BACKGROUND: Coronavirus disease 2019 is an infectious disease caused by severe acute respiratory syndrome virus coronavirus 2 (SARS-COV-2). Many aspects of its pathology and pathogenesis are not well understood. MATERIAL AND METHODS: We describe a series of spontaneous air leak cases we found in our coronavirus disease 2019 (COVID-19) positive 1086-patient cohort. RESULTS: Two out of six patients eventually required mechanical ventilation and succumbed to COVID-19. We presume that acute lung injury leading to SARS-CoV-2 with associated acute respiratory distress syndrome predisposes patients to this complication. CONCLUSION: This series is presented to highlight the emerging association of COVID-19 with spontaneous air leaks leading to pneumomediastinum, pneumothorax, and subsequent subcutaneous emphysema even in patients who have never received invasive mechanical ventilation and this may be more likely with the institution of high flow nasal cannula.

A rare constellation of eventration of right diaphragm, severe right lung hypoplasia, and LPA sling.

Left pulmonary artery sling (LPAS) is a rare congenital cardiac anomaly and is commonly associated with complete tracheal rings and various degrees of tracheobronchial compression. We present a very rare association of LPAS and congenital eventration of right hemidiaphragm in this case report.

Hypoplastic left heart syndrome with anomalous origin of right pulmonary artery.

Classic hypoplastic left heart syndrome is a rare but fatal congenital heart disease associated with variable underdevelopment of the left side of the heart. Complex forms of hypoplastic left heart syndrome have been reported to coexist with anomalous pulmonary venous drainage, transposition of the great arteries, or pulmonary valve dysplasia. We report a case of hypoplastic left heart syndrome with anomalous origin of the right pulmonary artery from the ascending aorta a rare association not reported in the literature. Preoperative comprehensive echocardiography is essential for diagnosis and accurate recognition of such rare anatomic variations.

Ascending Aortic Aneurysm in a Case of Arterial Tortuosity Syndrome: A Rare Tortuous Disorder.

Aortic aneurysms are a common entity among adults but very rare in the pediatric age-group. Association with autosomal inheritance is well established. We describe the unusual clinical presentation of a large ascending aortic aneurysm in a young child who was ultimately found to have severe diffuse arterial tortuosity.

Mid-Term Outcome of Right Ventricle to Pulmonary Artery Shunt for Older Children and Young Adults With Ventricular Septal Defect, Pulmonary Atresia, and Hypoplastic Pulmonary Arteries.

Management strategy for patients of ventricular septal defect and pulmonary atresia (VSD/PA) with hypoplastic pulmonary arteries presenting in late childhood or adolescence is still controversial. We present our experience with the use of right ventricle-pulmonary artery shunt (RV-PA) in management of this entity. Between January 2014 and April 2018, 25 patients of VSD/PA underwent valveless RV-PA shunt at our center. The size of the RV to PA shunt was calculated as half the expected diameter of the main pulmonary artery. We retrospectively reviewed the data from hospital records. Follow-up data were recorded from outpatient records or via telephone. Mean age of the cohort was 12.25 ± 3.18 years. There was 1 early and 1 interstage mortality. None of the patient developed acute renal failure, ventricular dysfunction, and arrhythmias. At interstage follow-up of 8.28 ± 3.7 months, both Nakata index (from 66.23 ± 24.12 to 185.8 ± 58 mm2/m2) and McGoon ratio (0.9 ± 0.22 vs 49 1.84 ± 0.4) increased significantly compared to preoperative value, whereas RPA-LPA ratio was not significantly changed (1.095 ± 0.39 vs 1.01 ± 0.56, P = 0.63). Prerepair pulmonary vascular resistance in 17 patients, who underwent complete repair, was 2.9 ± 0.69 woods unit/m2. Postrepair right ventricle-left ventricle pressure ratio was 0.5 ± 0.14. There was no early or late mortality and none of the patient required conduit revision or VSD fenestration. On follow-up of 25.75 ± 17.94 months, 16 patients were in NYHA I and 1 patient was in NYHA II. Appropriate-sized RV-PA shunt is an effective strategy for achieving balanced pulmonary artery growth in VSD/PA with hypoplastic pulmonary arteries presenting late without the risk of pulmonary over circulation or systemic malperfusion.

Direct Femoral Cannulation in Minimal Invasive Pediatric Cardiac Surgery: Our Experience With Midterm Result.

OBJECTIVE: One of the major challenges faced in minimally invasive pediatric cardiac surgery is cannulation strategy for cardiopulmonary bypass. Central aortic cannulation through the same incision has been the usual strategy, but it has the disadvantage of cluttering of the operative field. We hereby present the results of femoral cannulation in minimally invasive pediatric cardiac surgery in terms of adequacy and safety. METHODS: From January 2013 to June 2016, 200 children (122 males) with mean ± SD age of 9.2 ± 4.51 years (median = 6 years, range = 3-18 years) and weight of 19.22 ± 8.49 kg (median = 15 kg, range = 8-45 kg) were operated for congenital cardiac defects through anterolateral thoracotomy. The most common diagnosis was atrial septal defect (144 patients). In all the patients, femoral artery and femoral vein were cannulated along with direct superior vena cava cannulation for institution of cardiopulmonary bypass. RESULTS: There were no deaths or any major complications related to femoral cannulation. Femoral artery cannulation provided adequate arterial inflow, whereas femoral vein with direct superior vena cava cannulation provided adequate venous return in all the patients. No patient required vacuum-assisted venous drainage. No patient required conversion to sternotomy or developed vascular, neurological complications. At discharge and at 1-year follow-up, both femoral artery and vein were patent without a significant stenosis on color Doppler ultrasonography in all the patients. At mean ± SD follow-up period of 30.63 ± 10.09 months, all the patients were doing well without any wound-related, neurological, or vascular complications. CONCLUSIONS: Femoral arterial and venous cannulation is a feasible, reliable, and efficient method for institution of cardiopulmonary bypass in minimally invasive pediatric cardiac surgery.

Multiple left ventricular aneurysms in a young female.

Multiple left ventricular aneurysms (LVAs) are rare, especially in a young female. A 29-year-old woman presented vague symptoms. Multiple LVAs were revealed and confirmed on different imaging modalities, including chest radiography, echocardiography, contrast ventriculography and cardiac magnetic resonance imaging. Detailed work-up for probable etiologies including ischemic, infectious, inflammatory and autoimmune causes was negative. In the absence of angina, decompensated congestive heart failure, arrhythmias and embolism, the patient was managed conservatively, with excellent mid-term outcome.

Total anomalous systemic and pulmonary venous connection.

Total anomalous systemic and pulmonary venous connection is an extremely rare congenital cardiac anomaly. We present our unique experience of managing this complex partially diagnosed cardiac anomaly in a 16-month-old boy. The systemic venous anomaly was not detected during the initial preoperative evaluation. He was doing well on follow-up, with normal pulmonary artery pressure.