BackgroundTetralogy of Fallot (TOF) is a cyanotic disease requiring early intervention. We assessed the effect of right ventricular outflow tract (RVOT) stenting versus modified Blalock-Taussig shunt (mBTS) on outcomes of surgical repair of TOF.MethodsFifteen palliated TOF infants underwent complete repair surgery. RVOT stenting was performed in seven infants and mBTS was done in eight infants. Data on sequential patients who underwent surgery were collected and reviewed retrospectively.ResultsStenting group were significantly younger (1.62 ± 0.34 vs 2.80 ± 0.52, p = 0.001), had lower body weight (3.28 ± 0.48 vs 5.03 ± 0.67, p = 0.001) and lesser body surface area (0.20 ± 0.01 vs 0.26 ± 0.20, p = 0.001) than the mBTS group at palliation. Mean right pulmonary artery (RPA) diameter in stenting group at palliation was 2.9 ± 0.54 mm (z-score -3.08 ± 0.97) and increased at surgery to 4.6 ± 0.49 mm (z-score –0.79 ± 0.66) (p = 0.001). The mean left pulmonary artery (LPA) diameter was 2.5 ± 0.42 mm (z-score -3.3 ± 0.86), which increased to 3.3 ± 0.40 mm (z-score -2.2 ± 0.74) at surgery (p = 0.005). The mean RPA diameter in mBTS group at palliation was 3.2 ± 0.32 mm (z-score –2.9 ± 0.70) and increased at surgery to 4.3 ± 0.55 mm (z-score –1.1 ± 0.94) (p = 0.001). The mean LPA diameter was 2.8 ± 0.26 mm (z-score -3.3 ± 0.62), which increased to 3.2 ± 0.24 mm (z-score –2.4 ± 0.52) at surgery (p = 0.032). Repeat echocardiography showed significant increase in McGoon ratio and Nakata index in both groups (p = 0.001). No significant differences were seen between the two groups regarding surgical procedure and postoperative complications.ConclusionRVOT stenting is a safe and effective approach instead of mBTS in hazardous TOF infants with hypercyanotic spell, small PAs and complex anatomies.

Rationale:Primary cardiac lymphoma (PLC) is an extremely uncommon malignancy. PCL is more common in secondary immunodeficient patients. In this report, we describe a unique case of PLC who had been diagnosed as a STK4 deficient patient. This case is the first Primary immunodeficiency (PID) patient developing PCL in the world.Patient concerns:An eleven-year-old girl, a known case of PID, was referred to the pediatric cardiology department because of chest pain and dyspnea. Her CXR revealed cardiomegaly without mediastinal involvement and the echocardiography showed a mild pericardial effusion and cystic-shape echogenic masses.Diagnoses:After a period of missed follow up, she presented with respiratory distress following with syncope at the clinic because of a pressure effect of a large mass on the right ventricular outflow tract (RVOT) .An emergency operation was done for debulking of the tumors and resolving of RVOT obstruction. Biopsy and immunohistochemical staining was revealing “T-cell lymphoma”, non-Hodgkin's type.Interventions:Chemotherapy was done with cyclophosphamide, methotrexate, adriamycine, vincristine, hydrocortisone and allopurinol.Outcomes:The tumors shrank after chemotherapy initiation and she stayed stable for almost one month. Finally, she developed sever thrombocytopenia during her chemotherapy and died because of lung hemorrhage two months after her operation.Lessons:Although PCL is very rare, it must be considered in the differential diagnosis of intracardiac mass or refractory pericardial effusions, especially in PIDs which are widely known for developing EBV-associated diseases such as lymphoma.

We herein describe a case series of children with SARS-CoV-2 infection (COVID-19) complicated with acute intracardiac thrombosis. The diagnosis of COVID-19 was confirmed through the reverse transcription-polymerase chain reaction (RT-PCR). Transthoracic echocardiography of patients revealed large intracardiac mobile masses resected successfully via cardiac surgery. The underlying mechanisms of this thrombus in the COVID-19 infection may be attributed to the hypercoagulation and inflammatory state of the disease incurred by the SARS-CoV-2 virus.

Introduction: Most of the ventricular septal defects (VSD) are complicated with pulmonary arterial hypertension (PAH) which is the major cause of pulmonary hypertensive crisis and right ventricular failure. Methods: We reviewed clinical outcomes of 63 infants who underwent cardiac surgery and were divided into three groups. Control group (n=20) did not received sildenafil while group A (n = 22) received drug (0.3 mg/kg) before and after surgery. Group B (n=21) received drug at the initiation of surgery. Demographic data, preoperative and postoperative variables were compared among the patients. Results: Patients in the group A had lower preoperative pulmonary arterial pressure (PAP) compared to other groups (P < 0.001). Also, patients in control group had longer cardiopulmonary bypass time (P < 0.05). Postoperative PAP in patients of group A and B decreased significantly compared to control group (P < 0.001). Also, pre- and postoperative PVR (pulmonary vascular resistance) showed a significant decrease in group A compared with control and group B (P < 0.001). The intubation time in patients of the control group was significantly more prolonged compared with patients of group A and B (P < 0.001). Moreover, the length of ICU stay was significantly longer in patients of control group compared with group A and B (P < 0.001). Conclusion: Preoperative sildenafil therapy seems to be effective and safe to prevent postoperative PAH and pulmonary hypertensive crisis in children with ventricular septal defects and has a positive impact on postoperative care.