Novel echocardiographic score to predict duct-dependency after percutaneous relief of critical pulmonary valve stenosis/atresia.

OBJECTIVES: This study aimed to identify clinical, hemodynamic, or echocardiographic predictive features of persistent duct-dependency of pulmonary circulation (PDDPC) after effective percutaneous relief of pulmonary atresia with the intact ventricular septum (PA-IVS) or critical pulmonary stenosis (CPS). METHODS: From 2010 to 2021, 55 neonates with PA-IVS or CPS underwent percutaneous right ventricle (RV) decompression at our Institution. After successfully relief of critical obstruction, 27 patients (group I) showed PDDPC, whereas RV was able to support the pulmonary circulation in the remaining 28 patients (group II). Clinical, hemodynamic, and echocardiographic features of these two groups were compared. RESULTS: No significant difference in clinical and hemodynamic data was found between the groups, although the group I had a lower oxygen saturation at hospital admission. However, tricuspid valve (TV) diameter <8.8 mm, TV z-score ←2.12, tricuspid/mitral valve annular ratio <.78, pulmonary valve diameter <6.7 mm, pulmonary valve z-score ←1.17, end-diastolic RV area <1.35 cm2 , end-systolic right atrium area >2.45 cm2 , percentage amount of interatrial right-to-left shunt >69.5%, moderate/severe tricuspid regurgitation, RV systolic pressure >42.5 mmHg, tricuspid E/E' ratio >6.6 showed each significant predictive value of PDDPC. These parameters were used to build a composite echocardiographic score (PDDPC-score), assigning one point each above the respective cut-off value. A score ≥4.00 showed high sensitivity (100%) and specificity (86%) in predicting PDDPC. CONCLUSION: Clinical and hemodynamic features fail to predict the short-term fate of the pulmonary circulation after successful treatment of PA-IVS/CPS. However, a simple, composite echocardiographic score is useful to predict PDDPC and could be crucial in the management of this frail subset of patients.

Rare case of Kawasaki disease with cardiac tamponade and giant coronary artery aneurysms.

Kawasaki disease is an acute systemic vascular disease, generally self-limited and typically affecting children <5 years old, which leads to coronary artery aneurysms in about 25% of untreated cases. Cardiovascular involvement is characterised by transient pancarditis, in acute phase, while coronary illness, ranging from mild dilation to giant CAAs occurs late, rarely before the 10th day since fever onset. Here, we describe a peculiar case of KD, which occurred in a 4-month-old infant and presented with exudate cardiac tamponade and early giant aneurism of both the proximal right coronary artery) and the left circumflex coronary artery, in acute phase of the disease.