Prenatally Diagnosis and Outcome of Fetuses with Cardiac Rhabdomyoma – Single Centre Experience

  • Ramush Bejiqi Division of Cardiology, Pediatric Clinic, University Clinical Center of Kosovo, Prishtina, Kosovo
  • Ragip Retkoceri Division of Cardiology, Pediatric Clinic, University Clinical Center of Kosovo, Prishtina, Kosovo
  • Hana Bejiqi Main Center of Family Medicine, Prishtina, Kosovo
Keywords: cardiac rhabdomyoma, fetal echocardiography, tuberous sclerosis complex

Abstract

BACKGROUND: Cardiac rhabdomyoma (CRs) are the most common primary tumour of the heart in infants and children. Usually are multiple and, basing on the location can cause a haemodynamic disturbance, dysrhythmias or heart failure during the fetal and early postnatal period. CRs have a natural history of spontaneous regression and are closely associated with tuberous sclerosis complex (TSC). It has an association with tuberous sclerosis (TS), and in those, the tumour may regress and disappear completely, or remain consistent in size.

AIM: We aimed to evaluate the prenatal diagnosis, clinical presentation and outcome of CRs and their association with TSC in a single centre. The median follow-up period was three years (range: 6 months - 5 years).

MATERIAL AND METHODS: We reviewed medical records of all fetuses diagnosed prenatally with cardiac rhabdomyoma covering the period January 2010 to December 2016 which had undergone detailed ultrasound evaluation at a single centre with limited technical resources.

RESULTS: Twelve fetuses were included in the study; mostly had multiple tumours and a total of 53 tumours were identified in all patients - the maximum was one fetus with16 tumours. All patients were diagnosed prenatally by fetal echocardiography. In two patient's haemodynamic disturbances during the fetal period was noted and pregnancies have been terminated. After long consultation termination of pregnancy was chosen by the parents in totally 8 cases. In four continuing pregnancies during the first year of live tumours regressed. TSC was diagnosed in all patients during the follow-up.

CONCLUSIONS: Cardiac rhabdomyoma are benign from the cardiovascular standpoint in most affected fetuses. An early prenatal diagnosis may help for an adequate planning of perinatal monitoring and treatment with the involvement of a multidisciplinary team. Large tumour size, the number of tumours and localisation may cause hydrops, and they are significantly associated with poor neonatal outcome.

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Author Biography

Ramush Bejiqi, Division of Cardiology, Pediatric Clinic, University Clinical Center of Kosovo, Prishtina, Kosovo
Ramush Bejiqi was born in 1961, in Rama Baje, Medvedja, Yugoslavia. He has graduated medicine 1981-1986 at the Medical Facality in Nish, Serbia, where he completed Master of Scineces in imunology and alergology (1986 – 1988).  He also finished his pediatric specialisation (5 years) in Nish, Serbia (1991 – 1995), as well as subspecialization in cardiology (2 years) in Belgrade, Serbia (1995 – 1997).  He defended his PhD in area of congenital heart disease,  titeled: “Congenital Heart Disease in Neonates with Central Cyanosis†in Tirana, Albania (2010). He was trained in transthoracic echocardiography, transesophageal echocardiography, 3D, 4D; fetal echocardiography; interventional pediatric cardiology; and abdominal echosonography.  He started his professional carrea in 1986-1991 at Medical Center, Leskovac, Medvedja, Serbia. From 1991 and onward he is employed at the Pediatric Clinic, University Clinical Center of Kosovo,  Prishtina, Kosovo. From March, 2010 he is President of Pediatric Association of Republic of Kosovo. He is Member of European Association of Pediatric Cardiologist; Member of  American College of Cardiology;  Fellow of American College of Cardiology, New Orelans, USA; Member of Editorial Board of Medical Journal “Medicusâ€,  Republic of Macedonia. In March 2011 he was elected as a Director of Pediatric Emergency in Prishtina;  in September, 2011 he was elected as a Director of Board for developing Cardiac Surgery Center in Prishtina; and Director of 1st Pediatric School of Pediatric Association of Republic of Kosovo.

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Published
2017-03-20
How to Cite
1.
Bejiqi R, Retkoceri R, Bejiqi H. Prenatally Diagnosis and Outcome of Fetuses with Cardiac Rhabdomyoma – Single Centre Experience. Open Access Maced J Med Sci [Internet]. 2017Mar.20 [cited 2020Oct.30];5(2):193-6. Available from: https://www.id-press.eu/mjms/article/view/oamjms.2017.040
Section
B - Clinical Sciences

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