Background
Transposition of the great vessels is a congenital cyanotic heart defect. The hallmark of transposition of the great arteries is ventriculoarterial discordance, in which the aorta arises from the morphologic right ventricle and the pulmonary artery arises from the morphologic left ventricle. The Rastelli operation is performed for the repair of d-transposition of the great vessels with ventricular septal defect and pulmonary stenosis. During the surgery right ventricular outflow truckt – pulmonary arteries conduit is implanted and VSD is closed by patch.
Case presentation
A 22-year-old men with Transposition of great arteries, ventricular septal defect and pulmonary stenosis. At the age of 5 years surgical correction modo Rastelli was performed, at the age of 9 he was reoperated because of stenosis of right ventricle to pulmonary artery conduit and LVOT stenosis. On admission to our department he was in general good condition with no symptoms. Physical examination revealed no enlargement of the liver no odema, systolic murmur under pulmonary valve 3/6. Laboratory studies have found: E – 5580000, Hg- 15.9 g/dl, Ht- 45.6%, ASPAT- 21 U/L, ALAT- 20 U/L. Holter monitoring did not reveale significant arrhythmias. Cardiopulmonary exercise test showed very good exercise tolerance with VO2peak 25.7 ml/kg/min. Echocardiography showed: normal right ventricle function, LVOT gradient 30/21 mm/Hg, RV-PA graft gradient about 70 mmHg, no tricuspid regurgitation. CT did not find calcification and narrowing in RV- PA graft. The patient was qualify to further observation.
Current guidelines
Indications for intervention in patients with right ventricular to pulmonary artery conduits:
Symptomatic patients with RV systolic pressure >60 mmHg (TR velocity >3.5 m/s; may be lower in case of reduced flow) and/or moderate/ severe PR should undergo surgery I C.
Asymptomatic patients with severe RVOTO and/ or severe PR should be considered for surgery when at least one of the following criteria is present IIa C:
• Decrease in exercise capacity (CPET)
• Progressive RV dilation
• Progressive RV systolic dysfunction
• Progressive TR (at least moderate)
• RV systolic pressure >80 mmHg (TR velocity
>4.3 m/s)
• Sustained atrial/ventricular arrhythmias
1.ESC Guidelines for the management of grown-up congenital heart disease. European Heart Journal (2010) 31, 2915–2957
Expert’s comments:(Written authorization required from each expert)
1. Prof. Skalski J., MD, PhD
The patient is asymptomatic with normal RV-PA graft. There is no indication for surgery.
2. Prof. Lesław Szydłowski MD, PhD
Doppler measurements may be unreliable in mesurment RV-PA gradient. TR velocity with estimation of RV pressure should always be used in addition when assessing severity.
Expert’s conclusions:
Because of normal RV-PA graft the patient was qualified for observation.
Authors
Lidia Tomkiewicz-Pająk MD. PhD.1, Maria Olszowska MD. PhD.1, Prof. Piotr Podolec MD. PhD.1
Experts
Prof. Skalski J., MD, PhD, Prof. Lesław Szydłowski MD, PhD
1Department of Heart and Vascular Disease, John Paul II Hospital, Kraków, Poland
