8cm (arrow head) and an ASD (arrow). MAPCA from the descending aorta (right). Note the absent pulmonary trunk, right ventricular hypertrophy and multiple, … Fig. 2. Axial contrast-enhanced CT images showing left upper lobe pulmonary artery with filling defects representing wall adherent thrombi (arrow head) and reactive hilar lymphadenopathy (arrow, upper left), multiple peripherally located pulmonary screening library lesions (upper … Echocardiography revealed an ostium secundum type ASD, a VSD, a moderately reduced left ventricular ejection fraction (45%), and two large vegetations on the tricuspid valve. Blood cultures were positive for staphylococcus aureus. Targeted antibiotic treatment was initiated immediately, and the patient was referred to the cardiac surgery department for tricusipid valve repair which was performed 2 days after admission.
Due to a protracted clinical course and neurologic deterioration after surgery a cranial CT was performed. Several hypoattenuating cerebral and cerebellar lesions were predominantly localized in the right hemisphere consistent with thromboembolic events (Fig. 3). The further course of the patient was unfavorable, showing progressive cardiorespiratory insufficiency and increasing demand for vasoactive agents. Eighteen days after initial admission the patient passed away, due to cardiopulmonary failure. Fig. 3. Non-enhanced cranial CT images showing hypoattenuating lesions in the left cerebral peduncle, in the right occipital lobe, thalamus, and cuneus (arrows), consistent with embolic infarctions. Discussion TOF is one of the most common congenital heart malformations.
It consists of an interventricular communication, a biventricular connection of the aortic root, which overrides the muscular ventricular septum an obstruction of the right ventricular outflow tract, and RVH (1). The variants of TOF include TOF with a patent foramen ovale/ASD (pentalogy), TOF with absent pulmonary valve, and TOF with pulmonary atresia (Fig. 4). Fig. 4. The four TOF-defining components (left) and three most common variants of TOF (right). TOF plus PFO/ASD also referred to as pentalogy of Fallot. TOF with pulmonary atresia is clinically and radiologically distinct from regular TOF. It comprises 5�C10% of all tetralogy complexes (2). The anatomy of central pulmonary arteries is often abnormal (3,4).
MAPCA are systemic-to-pulmonary collateral arteries representing remnants of the embryonic ventral splanchnic arteries and provide an alternative pulmonary blood supply in patients with TOF and pulmonary atresia. These embryonic vessels normally regress concomitantly with the formation of the normal pulmonary arterial system in the first weeks of gestation, whereas in patients with pulmonary atresia they persist (5). Survival rates in TOF with pulmonary atresia without surgical repair reported in the literature are as low as 50% at 1 year of age Dacomitinib and 8% at 10 years (6).
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