63. Pulmonary atresia with intact ventricular septum, associated with intracranial calcifications and left parietal hemangioma (Sturge–Weber Syndrome)

Case reportNeurocutaneous disorders are characterized by skin lesions (port wine stain, hypopigmented patches) associated with intracranial features (ipsilateral leptomeningeal angiomas, intracranial calcifications and subsequent seizures). Some of these patients will also have opthalmological findings.These disorders are occasionally accompanied by congenital heart disease. Example: PHACE syndrome which is associated with aortic coarctation. On the contrary, Sturge–Weber syndrome, apart from one single case report, is usually not described with congenital heart disease. In the mentioned case report, 13year old female had along with this syndrome, pulmonary atresia with ventricular septal defect. As the author of this case report questioned whether this might be a new association, we would like to report our patient.This is a 14months old boy with pulmonary atresia/intact ventricular septum along with intracranial calcification, seizures along with strabismus and tunnel vision. Clinical findings were similar to Sturge–Weber syndrome, though cutaneous features were not present. Brain CT angio with contrast was diagnostic for Sturge–Weber and showed calcifications as well as left parietal hemangioma.As far as we know, this specific association has never been reported before and might be a new constellation as suggested by Huseyin Tan et al. (2003)

Giant right coronary artery aneurysm secondary to Kawasaki disease in an infant

Kawasaki disease (KD) is a potentially decapacitating multisystemic vasculitis with unknown etiology that acquired worldwide attention due to associated coronary aneurysms leading to life-threatening complications in very young babies including thrombosis, ischemia, and rupture. High levels of suspicion for early diagnosis and prompt treatment are crucial in preventing serious complications. We report here one of the patients who developed a giant coronary aneurysm but fortunately not a life-threatening complication after 5 years of follow-up. We conclude that later intravenous immunoglobulins (IVIG) treatment could be an important factor—among others—that precipitate into such complications. Keywords: Coronary artery, Echocardiogram, Giant coronary artery aneurysm, Infant, Kawasaki diseas

Impact of Interatrial Communication on Left Ventricle Performance in Patients with Significant Post-tricuspid Shunt.

Infants with post-tricuspid valve shunts (PTS) may benefit from interatrial communication (IAC). The effect of IAC on left ventricular (LV) performance in these patients was studied. IAC was documented prospectively in 55 patients with PTS. Clinical status, echocardiographic dimensions of LV, mitral inflow Doppler, tissue Doppler velocities and time intervals were measured. Creatinine kinase (CK), CKMB, troponin-I and NT pro-brain natriuretic peptide (NT pro-BNP) were measured. Patients were divided into four groups: (A) PTS but no IAC (n = 32); (B) PTS and IAC (n = 23); (C) VSD but no IAC (n = 16); and (D) VSD and IAC (n = 19). Group A had more frequent mitral regurgitation (p = 0.041), larger mitral annulus (1.80 vs. 1.30 cm, p < 0.0001) and larger LV systolic and diastolic dimensions (2.01 vs. 1.40 and 3.28 vs. 2.35 cm, p < 0.001) than group B. The E-wave deceleration time tended to be longer in group A (121.0 vs. 106.8 ms, p = 0.06). By tissue Doppler, group A had E'- and S-waves significantly taller (15.51 vs. 13.14 and 7.69 vs. 6.72 cm, p = 0.04 and p = 0.005, respectively) than group B. Also, NT pro-BNP was significantly higher in group A (1116.15 vs. 458.73 pg/ml, p = 0.028). Group C had significant larger mitral z-score values (1.2 vs. 0.01, p < 0.001), larger LV diameter z-score (p = 0.001) and higher NT pro-BNP level (1477.37 vs. 451.66 pg/ml, p = 0.001) than group D. There was no significant difference in the clinical status between the groups. In children with PTS, the presence of IAC could be beneficial. Their echocardiographic parameters and biomarker show better systolic and diastolic LV performance