Background: Malignant cardiac neoplasms are extremely rare in neonates. Prenatal diagnosis is often not available. Initial symptoms can mimic non-cardiac diseases. We present a pre-mature newborn, birth-weight 2,480 g, 34 gestational weeks, which underwent cardiac surgery due to a cardiac tumor.Case Summary: This was a 3–rd pregnancy after two spontaneous abortions to a mother with thrombophilia, diabetes, hydramnios, and retroplacental hematoma. The baby was admitted to NICU with transitory respiratory failure and inborn infection; hence oxygen-supplementation and antibiotics were initiated. On day 11 a deterioration with tachypnea, high oxygen requirements, significantly increasing C-reactive protein values were noted. Chest radiographs were unremarkable. On day 18 a life-threatening condition with clinical symptoms of shock was identified. Echocardiography showed a large tumor formation in the right atrium, reduced blood flow in the right ventricle and pulmonary artery. On day 19 cardiac surgery was performed: a large tumor with a myxomatous appearance that occupied the cavity of the right atrium and infiltrated the annulus of the tricuspid valve was extirpated. The front wall was restored with a patch. Hemodynamics was temporarily stabilized. On the following day, ventricular fibrillation and asystole occurred. Despite life support efforts, the outcome was lethal. The histological result confirmed leiomyosarcoma of the right atrium and right ventricular hypotrophy.Conclusion: Inborn cardiac sarcomas are extremely rare. The prognosis is poor. Due to fast progression in the third trimester, they can be missed by fetal echocardiography in earlier pregnancy. The postnatal clinical course is aggressive, not specific until invasive infiltration or obstruction by the tumor mass occurred. In our case, the sarcoma mimicked an inborn infection, followed by nosocomial infection and septic shock. Despite low incidence, cardiac tumors should be kept in mind and echocardiography should be conducted if there are unclear symptoms with progressive deterioration during the neonatal period.
Accurate imaging of the morphology in a child with a rare and complex congenital heart disease (CHD) is crucial for the surgical planning and, if possible – the minimization of operative risk. We present a case of a 3-month-old child with a rare variant of Total Anomalous Pulmonary Venous Return (TAPVR) – mixed type – which illustrates the benefi t of additional imaging modalities and techniques for the operative planning. The patient’s anomalous drainage of the pulmonary veins (PV) is as follows: left and right lower PVs drained in the coronary venous sinus, left upper PV drained via a vertical vein into the innominate vein and the right upper PV drained into the superior vena cava (SVC). Clinical fi ndings consist of heart failure (HF), pulmonary venous congestion and mild hypoxemia developing within the fi rst days after birth. Transthoracic echocardiography (TTE) demonstrates the features of TAPVR, but the modality is unable to differentiate all of the described anatomic details. Accurate morphological diagnosis is obtained via contrast Computed Tomography (CT). The. additional techniques of 3D modelling and 3D printing of a patient-specifi c model, based on the acquired CT, contributed to the planning of the surgical strategy. The patient underwent a radical operation at age of 3 months. The unique aspect of the case is the application of all possible operative techniques for correction of TAPVR – coronary sinus deroofi ng, anastomosis of a draining vessel into the left atrial appendage and Warden procedure which involves tunnelling of the right upper PVs from the SVC into the left atrium. Feedback from the surgical team shows that the additional imaging methods and the printed 3D model represent the pathology in detail that completely corresponds to the intra-operative fi ndings
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