*Corresponding author:Giovanni Battista Luciani, Division of Cardiac Surgery, Department of Surgery, University of Verona, Italy
Received: July 19, 2018; Published: July 26, 2018
To view the Full Article Peer-reviewed Article PDF
Background: Primary cardiac tumors (PCT) in children are rare disorders. Prenatal diagnosis is feasible. Most of them have favorable prognosis, but some still prove fatal.Objective: To define clinical outcome of benign PCT and the possible impact of prenatal diagnosis.
Methods: 14 cases were collected between December 2005 and August 201. We included patients with pre and postnatal diagnosis, medically and surgically managed. Pre and postnatal examinations, surgical procedures, clinical information before and after surgery, histological diagnoses were reviewed.
Results: Seventy-two percent of PCT were detected prenatally. Median follow-up time was 3,13 years. In the medical managed group, one had complete spontaneous regression, 3 had spontaneous reduction, 2 underwent efficacious Enviroximes treatment and one was lost to follow-up. 46% of cases needed surgery. In two infants, perioperative support with V-A ECMO and iNO was necessary. Complete excision of the mass was possible in 5 cases. Rate of hospital death was 20%. All but one late survivors were symptom-free at last clinical assessment. Clinical relevant recurrence of mixoma occurred in one case.
Conclusion: Benign PCT have extremely diverse course, some proving fatal in spite of benign histology. Prenatal diagnosis is crucial to plan delivery and neonatal management, as some benign PCT require intensive care. Complete excision of the mass usually is feasible but may be limited by young age and extent of cardiac infiltration. Even if patients are generally well after surgery, long term follow up is mandatory as some masses recur.
Keywords: Congenital Heart Disease; Primary Cardiac Tumors; Prenatal Diagnosis; Clinical Outcome
Abbreviations: PCT: Primary Cardiac Tumors; CHD: Congenital Heart Disease; FU: Follow Up; CT: Computed Tomography; CMRI: Cardiac Magnetic Resonance Imaging; NYHA: New York Heart Association; TOP: Termination of Pregnancy; SD: Standard Deviation; ECG: Electrocardiography; PGE: Prostaglandin; Ino: Inhaled Nitric Oxide; ASD: Atrial Septal Defect; VSD: Ventricular Septal Defect; V-A ECMO: Veno-Arterial Extracorporeal Membrane Oxygenation; CAT: Calcified Amorphous Tumor