This study investigates the role of oral sildenafil in decreasing pulmonary pressure after congenital heart surgery. Between September 2002 and September 2004, among a group of postoperative children with large septal defects, moderate to severe pulmonary hypertension [pulmonary artery (PA) to aortic (Ao) pressure ratio of 0.76 +/- 0.17] and systemic desaturation (Ao Sat = 0.89 +/- 0.11), oral sildenafil (0.3 mg x kg(-1), every 3 hours) was administered for a period of 24-48 hours (sildenafil group). These patients were compared to a group of 22 children with similar pathologies who did not receive sildenafil (control group). Postoperative PA pressure (28.61 +/- 7.80 vs 39.40 +/- 10.80 mm Hg) and PA/Ao pressure (0.28 +/- 0.08 vs 0.41 +/- 0.11) were significantly lower in the sildenafil group ( p = 0.001 and 0.001 respectively). Pulmonary hypertensive crisis was detected in 4 patients in the control group, but none in the sildenafil group ( p = 0.02). There was no significant rise in PA pressure following discontinuation of the drug (26.30 +/- 6.66 vs 28.49 +/- 10.93 mm Hg, p = 0.366). No significant complications were noticed regarding sildenafil use. Low doses of oral sildenafil appear to be effective and safe to control postoperative PA pressure in children. Absence of rebound pulmonary hypertension, availability, and low cost of the drug are considered as its major advantages.

Pulmonary arterial hypertension (PAH) is a progressive disease. In recent years, phosphodiesterase type 5 inhibitors such as sildenafil have been used to treat this disease in children. Recently, tadalafil has been used in adults with similar efficacy but it has been used less often in children. This experimental study was carried out in 18 known patients aged 4-24 years in the Emam Hossein Hospital of Isfahan, Iran. All patients had been taking sildenafil for a few months to years. Patients underwent echocardiographic study, the 6-minute walk test (6MWT), and non-invasive pulse oximetry before and after the 6MWT. These tests were repeated again after sildenafil had been switched to tadalafil for 6 weeks. After 6 weeks of tadalafil prescription, the severity of some of the patients' symptoms decreased, but the New York Heart Association class of the patients did not change more. Mean ± standard deviation (SD) oxygen saturation while taking sildenafil and after 6 weeks of tadalafil were significantly different (p = 0.005). Furthermore, mean ± SD oxygen saturation after the 6MWT while taking sildenafil and after 6 weeks of tadalafil were significantly different (p = 0.036). The mean ± SD distances walked in this test while taking sildenafil and tadalafil were significantly different (p = 0.005). No significant side effects were seen; 15 patients continued tadalafil. Tadalafil may be a safe drug to treat children and young adults with PAH. We did not observe any significant side effects during usage; it improves functional capacity and oxygen saturation better than sildenafil in these patients, and requires fewer daily doses than sildenafil.

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.

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.