Severe aortic stenosis can be accompanied by various degrees of left ventricular underdevelopment. The assessment whether a borderline-sized left ventricle can or cannot support the systemic circulation is crucial. The decision-making still remains challenging. We present a case that illustrates that the development of haemodynamic parameters can be difficult to estimate, even in the long term. The patient went from biventricular to univentricular circulation and back and could finally be palliated by heart transplantation. Modern technology including long-term mechanical cardiac support as a bridge to candidacy and drug therapy for pulmonary hypertension were vital to successfully combat a previously lethal disease.

Children's Heart Centre 2nd Faculty of Medicine Charles University Prague and Motol University Hospital 5 Uvalu 84 Prague 15006 Czech Republic

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