Type of study: Observational, longitudinal, retrospective, descriptive, single center cohort.
Current guideline recommendations indicate that repair in children with congenital heart disease (CHD) should be performed before irreversible pulmonary vascular disease (PVD) has developed. Accurate assessment of these patients is crucial. Previous work has shown that wedge pulmonary angiography (WPA) has correlated well with the severity of PVD and CHD pulmonary hypertension (PH-CHD).
We assessed the prognostic value of WPA in predicting in-hospital morbidity and long-term mortality in subjects with PH-CHD undergoing surgical repair.
We analyzed all children who underwent diagnostic cardiac catheterization and WPA at the National Institute of Cardiology in Mexico City from 2005 – 2017. 118 subjects with PH were included. WPA’s were evaluated by interventional cardiologists to determine general appearance, and determine the severity of vascular disease when present (Figure 1).
Subjects characteristics can be seen in Table 1. Subjects were classified as having normal WPA, abnormal unilateral WPA, or bilateral abnormal WPAs based on signs of PVD (Table 2). Hemodynamic characteristics can be seen in Table 3. Mortality rate was 8.25%. Estimated survival at 12 years after the surgery based on WPA abnormality was 97.2%, 95.6%, and 79.5%, respectively (p=0.049) (Figure 2). Similarly, survival based on baseline pulmonary vascular resistance (PVRI) (< 4, between 4-8, and > 8 WU) was also different between groups (p=0.043) (Figure 3). The prognostic survival significance of WPA was lost when the analysis was performed in patients with PVR between 4-8 WU (p=0.415). Baseline bilateral abnormal WPA had a risk ratio (RR) of 3.3 (CI 95% 1.1-10.4) to predict in-hospital composite morbidity index (heart failure, arrhythmias, and pulmonary hypertensive crisis).
WPA was able to predict long-term survival based on WPA extension of disease severity. This prognostic significance was lost in the group with PVR between 4- 8 WU. Bilateral abnormal WPA was able to predict postoperative morbidity.