Coronary changes and cardiac events in children diagnosed with kawasaki disease without initial coronary aneurysm: A multicenter retrospective cohort study.

Background: Kawasaki disease (KD) is a systemic vasculitis affecting young children, which may lead to coronary artery aneurysm (CAA). The optimal timing of serial echocardiography in patients with uncomplicated KD is debated. Objectives: To assess changes in coronary artery Z-scores from the initial diagnosis, two weeks, eight weeks, and one year following diagnosis and adverse cardiac events in children diagnosed with KD without initial CAA. Methods: Retrospective chart reviews of four referral centers in Thailand were conducted of all children who were diagnosed with KD without initial CAA (coronary artery Z-score < 2.5) between 2017 and 2020. Eligibility criteria included the absence of congenital heart disease and patients with available echocardiographic evaluations at baseline and at eight weeks of illness. The two-week and one-year echocardiographies were reported. Adverse cardiac events at one year from diagnosis were explored. The primary outcome was a maximal coronary Z-score on the follow-up echocardiography at eight weeks and one year. Results: Of 200 patients diagnosed with KD, 144 patients (72%) did not have CAA. A total of 110 patients were included in the study. The median age was 23 months (IQR, 2-39 months) and 60% were male. Fifty patients (45.5%) had incomplete KD, and four (3.6%) received a second intravenous immunoglobulin treatment. Of 110 patients, 26 patients (23.6%) had coronary ectasia (Z-score of 2-2.49) on their initial echocardiographic examination. Sixty-four patients were evaluated in two-week echocardiographic studies, which showed four new small CAAs and five coronary ectasia. At 8 weeks, 110 patients had undergone complete echocardiographic studies. No patient had residual CAAs. Only one patient had persistent coronary ectasia that regressed to normal within one year. At one-year follow-up (n = 90), no cardiac events were reported. Conclusion: New CAA in-patients with KD who had no previous CAA in their initial echocardiography are rare. In addition, patients who had normal echocardiographic follow-up at two weeks or eight weeks mostly continued to be normal at one year. The optimal timing of the echocardiographic follow-up should be at two to eight weeks in patients without initial CAA, who still have a coronary artery Z-score < 2 at the second echocardiography.Trial registration: TCTR20210603001.

Contemporary survival of patients with pulmonary arterial hypertension and congenital systemic to pulmonary shunts.

OBJECTIVE: To compare survival of patients with newly diagnosed pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) according to various clinical classifications with classifications of anatomical-pathophysiological systemic to pulmonary shunts in a single-center cohort. METHODS: All prevalent cases of PAH-CHD with hemodynamic confirmation by cardiac catheterization in 1995-2015 were retrospectively reviewed. Patients who were younger than three months of age, or with single ventricle following surgery were excluded. Baseline characteristics and clinical outcomes were retrieved from the database. The survival analysis was performed at the end of 2016. Prognostic factors were identified using multivariate analysis. RESULTS: A total of 366 consecutive patients (24.5 ± 17.6 years of age, 40% male) with PAH-CHD were analyzed. Most had simple shunts (85 pre-tricuspid, 105 post-tricuspid, 102 combined shunts). Patients with pre-tricuspid shunts were significantly older at diagnosis in comparison to post-tricuspid, combined, and complex shunts. Clinical classifications identified patients as having Eisenmenger syndrome (ES, 26.8%), prevalent left to right shunt (66.7%), PAH with small defect (3%), or PAH following defect correction (3.5%). At follow-up (median = 5.9 years; 0.1-20.7 years), no statistically significant differences in survival rate were seen among the anatomical-pathophysiological shunts (p = 0.1). Conversely, the clinical classifications revealed that patients with PAH-small defect had inferior survival compared to patients with ES, PAH post-corrective surgery, or PAH with prevalent left to right shunt (p = 0.01). Significant mortality risks were functional class III, age < 10 years, PAH-small defect, elevated right atrial pressure > 15 mmHg, and baseline PVR > 8 WU•m.2. CONCLUSION: Patients with PAH-CHD had a modest long-term survival. Different anatomical-pathophysiological shunts affect the natural presentation, while clinical classifications indicate treatment strategies and survival. Contemporary therapy improves survival in deliberately selected patients.