We present four children with hypoplastic left heart syndrome (HLHS) and development of thrombus in the native aortic root. There are only two previously reported cases in the literature. In all four of our patients, thrombus was noted on transthoracic echocardiography. In one patient, surgical thrombectomy was performed, with subsequent deterioration and death. Thrombolysis was used in one patient, with suspected embolic events following his treatment. In the remaining two patients, medical management with chronic anticoagulation was successful in reducing the size of the thrombus.
Background
Advancements in the field, including novel procedures and multiple interventions, require an updated approach to accurately assess patient risk. This study aims to modernize patient hemodynamic and procedural risk classification through the creation of risk assessment tools to be used in congenital cardiac catheterization.
Methods and Results
Data were collected for all cases performed at sites participating in the C3PO (Congenital Cardiac Catheterization Project on Outcomes) multicenter registry. Between January 2014 and December 2017, 23 119 cases were recorded in 13 participating institutions, of which 88% of patients were <18 years of age and 25% <1 year of age; a high‐severity adverse event occurred in 1193 (5.2%). Case types were defined by procedure(s) performed and grouped on the basis of association with the outcome, high‐severity adverse event. Thirty‐four unique case types were determined and stratified into 6 risk categories. Six hemodynamic indicator variables were empirically assessed, and a novel hemodynamic vulnerability score was determined by the frequency of high‐severity adverse events. In a multivariable model, case‐type risk category (odds ratios for category: 0=0.46, 1=1.00, 2=1.40, 3=2.68, 4=3.64, and 5=5.25; all
P
≤0.005) and hemodynamic vulnerability score (odds ratio for score: 0=1.00, 1=1.27, 2=1.89, and ≥3=2.03; all
P
≤0.006) remained independent predictors of patient risk.
Conclusions
These case‐type risk categories and the weighted hemodynamic vulnerability score both serve as independent predictors of patient risk for high‐severity adverse events. This contemporary procedure‐type risk metric and weighted hemodynamic vulnerability score will improve our understanding of patient and procedural outcomes.
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