Background
Truncus arteriosus communis (TAC) is a rare congenital heart disease appearing in 0.034 to 0.56 per 1,000 newborns and representing 1.4% to 2.8% of all cases of congenital heart disease . In this anomaly, a single arterial trunk arises from the heart, overrides the interventricular septum, and supplies systemic, pulmonary, and coronary circulations. Without surgical treatment, 80% of patients die within the first year of life, usually in early infancy. Repair of TAC during the neonatal and early infant period has become standard practice in many centers, with very good results (1,2,3).
Case presentation
The heart defect was diagnosed in the early childhood – common truncus with VSD. The patient was operated on 1988y – VSD closure and Dacron conduit implantation were performed. On 1997y – reoperation – conduit was widen by homograft patch. On 2000- ineffective percutaneus balloon angioplasty of pulmonary branch was performed. The patient was without symptoms in good general condition.
On 2008- The patient was admitted to our center. On admission patient was haemodynamically stable in functional class NYHA I. He didn’t complain on exercise capacity.
Transthoracic echocardiogram was repeated and a calcification of pulmonary conduit was observed. The pressure gradient in right ventricle flow track was 100-140 mmHg.
In 24 hour monitoring we detected single ventricle extrasystolies. In cardiopulmonary exercise test normal exercise capacity was observed. Test was interrupted after 14 min 52 sec due to limit of heart rate. The oxygen consumption was 22,3 ml/kg/min, no pathologies in ECG was observed. CT heart study showed a calcification of pulmonary conduit.
In catheterization of the right heart we observed the high right ventricle pressure – 139mmHg and elevated pressure into pulmonary graft – 120mmHg. On 2012 – the patients was admitted for the control examination – she complained heart palpitation. Holter monitoring of ECG showed 1600 ventricular extrasystolies. CPX showed lower VO2peak.
Current guidelines
Indication for intervention in patients with right ventricular to pulmonary artery conduits – (4):
1. Symptomatic patients with RV systolic pressure > 60 mmHg and/or moderate/severe PR should undergo surgery IC
2. Intervention in asymptomatic patients with severe RVOTO or PR should be considered for surgery when at least one of the following criteria is present:
• progressive RV dilatation
• decrease in exercise capacity
• progressive RV systolic dysfunction
• progressive TR
• important arrhythmias
• RV systolic pessure > 80 mmHg IIa C
References
1.Mavroudis C, Backer CL. Truncus arteriosusIn: Mavroudis C, Backer CL, editors. Pediatric cardiac surgery. 3rd ed.. St Louis: CV Mosby; 2003. pp. 339-352.
2.Bove EL, Lupinetti FM, Pridjian AK, et al. Results of a policy of primary repair of truncus arteriosus in the neonate J Thorac Cardiovasc Surg 1993;105:1057-1066.
3.Hanley FL, Heinemann MK, Jonas RA, et al. Repair of truncus arteriosus in the neonate J Thorac Cardiovasc Surg 1993;105:1047-1056.
4. ESC Guidelines for the management of grown-up congenital heart disease (new version 2010), European Heart Journal (2010) 31, 2931-2933.
Expert’s comments:(Written authorization required from each expert)
1. Bogusław Kapelak, MD, PhD
The patient should be qualify for surgery. Because of very high risk of operation percutaneus intervention should be considered.
2. Jacek Kołcz MD, PhD
There is a typical late complication after repair of common truncus arteriosus. The patient should be reopetrated.
Expert’s conclusions:
The patient after Common Truncus operation with the RV-PA conduit stenosis in case of high RV systolic blood pressure and decrease in exercise capacity she should be referred to a reoperation.
Authors:
Lidia Tomkiewicz-Pająk MD, PhD1 , Prof. Maria Olszowska MD, PhD1, Monika Komar MD, PhD1, Prof. Piotr Podolec MD, PhD1, Grzegorz Kopeć MD, PhD1, Jakub Podolec MD1.
Experts:
Bogusław Kapelak MD, PhD2, Jacek Kołcz MD, PhD3.
1Department of Cardiac and Vascular Disease in John Paul II Hospital, Institute of Cardiology, Faculty of Medicine, Jagiellonian University, Krakow, Poland.
2Department of Cardiac, Vessels Surgery and Transplantology, Jagiellonian University College of Medicine, John Paul II Hospital, Krakow, Poland
3Department of Pediatric Cardiac Surgeryy, Polish-American Children’s Hospital, Jagiellonian University, Krakow, Poland
