This year once again saw numerous scientific publications in the field of congenital heart disease (CHD). Much of the work stresses that, despite prognostic improvements, this population still faces significant morbidity and mortality.
Oliver et al.1 presented a study in a cohort of 3311 patients with CHD and investigate different risk factors for excess mortality. In this study, the annual mortality rate was 0.89% and the mean survival was 75.1 (95% confidence interval [95%CI], 73-77) years with a standardized mortality rate of 2.64 (95%CI, 2.3-3.0, P < .001). In multivariable analysis, 11 predictors of death from any cause were identified and patients with 1 or more risk factors had a significantly higher standardized mortality (5.22, 95%CI, 4.5-6.0; P < .001) than those without risk factors (1.14, 95%CI, 0.9-1.5, P = .19).
Another noteworthy work is that of Hjortshøj et al.,2 which described the current causes of death in patients with Eisenmenger syndrome. To do this, they followed 1546 patients from 13 countries between 1977 and 2015. During this period, 558 patients died (2.8% of patients per year), with heart failure (HF) the leading cause of death, followed by infectious etiology, sudden death, thromboembolism, hemorrhage, and periprocedural death. The authors analyzed the causes of death in 2 periods and observed a significant increase in mortality due to HF, as well as a decrease in mortality related to thromboembolism and procedures themselves. Overall, survival improved, with the average age of the deceased increasing from 36.9 ± 18.8 to 45.2 ± 16.2 years. In conclusion, patients died later and for chronic causes rather than acute events.
Crucially, HF is the end point of multiple CHDs, complicating its management, given the limited evidence on medical treatment, the difficulty of receiving a heart transplantation, and the lower probability of receiving ventricular support. This year, data were published on the use of mechanical ventricular assist devices in patients with CHD included in the INTERMACS registry,3 which includes more than 16 000 patients with long-term mechanical ventricular assist devices implanted in the United States; only 126 of the patients had been diagnosed with CHD, less than 1%. For the analysis, patients were divided into those with biventricular circulation and systemic morphologic left ventricle (n = 63), biventricular circulation and systemic morphologic right ventricle (n = 45), and univentricular circulation (n = 17). Compared with other etiologies, patients with CHD were younger and were more likely to require biventricular assistance (21% vs 7%) and, in most cases, assistance was used as a bridge to transplantation and not as destination therapy. The most interesting aspect of this work is that, although overall mortality was higher in patients with CHD, patients with CHD and biventricular circulation with a left ventricular assist device had the same mortality as patients without CHD, regardless of the systemic ventricular morphology. Among patients with biventricular assistance, mortality was higher in those with CHD, and the need for biventricular assistance was the only independent predictor of mortality in this population (hazard ratio [HR] = 4.4, 95%CI, 1.8-11.1).
Another of last year's hot topics in CHD was liver failure due to the Fontan circulation. Among the many relevant publications, the most interesting is possibly the review by Hilscher et al.,4 which details the clinical, analytical, imaging, and anatomopathological findings in the study of liver cirrhosis and its applicability to patients with the Fontan circulation. The authors recommend an annual clinical follow-up during the first 10 years after completion of the Fontan circulation, a complete analytical study including calculation of liver cirrhosis risk scores (the aspartate aminotransferase to platelet ratio index [APRI] and Fibrosis 4 [FIB-4]) every 2 or 3 years, and a liver ultrasound at 5 years. However, after 10 years, a complete annual assessment is recommended that includes a clinical and analytical evaluation and abdominal ultrasound. The presence of unexplained thrombocytopenia or ascites on ultrasound would be an indication for liver magnetic resonance imaging with elastography or a liver ultrasound with elastography to determine the degree of liver fibrosis. In the case of established cirrhosis, they propose liver ultrasound and a biannual alpha-fetoprotein determination to screen for hepatocellular carcinoma.
One of the diseases whose future has changed drastically is hypoplastic left heart syndrome (HLHS). Latus et al.5 analyzed patient outcomes according to palliation technique–Norwood surgery or the hybrid procedure–using magnetic resonance parameters after the second stage, prior to the Fontan procedure, and found no significant differences in right ventricular ejection fraction, preserved in both groups, or in cardiac output; however, strain values, intraventricular synchrony parameters, and pulmonary branch development were better after Norwood. The need for reinterventions also favored the post-Norwood group.