(IV-2A.1) Patient after total correction of Tetralogy of Fallot with syncope Magdalena Kostkiewicz MD, PhD, Sylwia Wiśniowska-Śmiałek MD, Agata Leśniak-Sobelga MD,PhD, Prof. Piotr Podolec MD, PhD



EXPERT: Tomasz Miszalski-Jamka, MD, PhD
Affiliation: Department of Radiology, John Paul II Hospital, Krakow, Poland
In CMR impaired RV contractility can be seen. Additionally pulmonary valve stenosis, accelerated blood flow and right pulmonary artery dilatation is present. Gradient in ostium is about 20 mmHg. There is no visible fibrosis in RV muscle.

EXPERT: Prof. Janusz Skalski MD, PhD
Affiliation: Department of Pediatric Cardiac Surgery, Jagiellonian University Medical College, Krakow
In this particular case residual heart defects seem insignificant, with no influence on hemodynamics. The question is if the clinical picture is stable or there is a progression. Stable picture tells us that patient is coping well with hemodynamic disturbances. In my opinion he needs observation, probably percutaneous pulmonary valve replacement is an option for him. Nevertheless percutaneous valve replacement might only be a temporally solution – after 10-15 years another replacement will probably be needed, this time surgical. At this point, I suggest, that pulmonary valve replacement should be performed when symptoms appear.

EXPERT: Assoc. Prof. Bogusław Kapelak MD, PhD
Affiliation: Department of Cardiovascular Surgery and Transplantology, John Paul II Hospital, Krakow
Optimal pharmacological treatment is in my opinion the most recommended option for this patient now. Pulmonary valve replacement means implantation of a biological valve with limited durability. In my opinion if replacement is considered, the intervention should be conducted when the symptoms occur. It is also important to remember, that solitary tricuspid valve repair without stenotic pulmonary valve replacement will be successful only for a short period of time.

EXPERT: Lidia Tomkiewicz-Pająk MD, PhD
Affiliation: Department of Cardiac and Vascular Diseases, John Paul II Hospital, Krakow, Poland
Enlargement of the RV and RA prompt the occurence of arrhythmia – especially atrial fibrillation. There is a significant pulmonary stenosis which causes further right chambers enlargement, fibrosis and continuous reduction of RV function. Therefore, patient needs close observation, beta blocker administration and cardiopulmonary exercise follow-up to estimate exercise capacity. Tricuspid regurgitation requires close observation as it may increase rapidly. In my opinion percutaneous pulmonary valve replacement should be considered.

EXPERT: Prof. Andrzej Rudziński MD, PhD
Affiliation: Department of Cardiology, Pediatric Institute, Jagiellonian University Medical College, Krakow, Poland
RV ejection fraction is 40%, RV volume – 400 ml, assuming average height it’s 220 ml/m2. According to guidelines volume of RV over 180 ml/m2 indicates severe ventricle disfunction. Together with tricuspid regurgitation fraction 30%, those are standard indicators for pulmonary valve replacement, not necessarily at present, but in close future.

EXPERT: Prof. Magdalena Kostkiewicz MD, PhD
Affiliation: Department of Cardiac and Vascular Diseases, John Paul II Hospital, Krakow
This patient has been under our supervision for the 6 years now. The clinical picture is stable. Circumstances of syncope aren’t clear. If pulmonary valve replacement is taken under consideration, percutaneous intervention seems the best option.

EXPERT: Prof. Lesław Szydłowski MD, PhD
Affiliation: 1st Department of Paediatric Cardiology, Medical University of Silesia, Katowice-Ligota, Poland
Close observation and follow-up is necessary in this patient. Although the gradient on pulmonary valve is now about 20 mmHg, it may raise with time and the pulmonary valve stenosis progress In my opinion when stenosis of pulmonary trunk is present, catheterization and probably balloon extension may be indicated.


1) Close observation with regular exercise tolerance evaluation – cardiopulmonary exercise test, repeated CM, Cardiac echo, Holter ECG is needed.
2) Consider pulmonary valve replacement – surgery or percutaneous intervention, when symptoms exacerbate.

“Development of the European Network in Orphan Cardiovascular Diseases”
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