Impact of severe valvular heart disease in adult congenital heart disease patients

Figures

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  FIGURE 1. Study flow-chart. ACHD, Adult congenital heart disease.

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  FIGURE 2. Distribution of severe heart valve lesions. (A) Among patients with severe valvular heart disease (S-VHD), 90 (89%) had single S-VHD (= 1 valve with severe dysfunction), while 11 (11%) had multiple S-VHD (≥ 2 valves with severe lesions). (B) Right-sided S-VHD (63 patients, 63%) were prevalent as compared to left-sided S-VHD (31 patients, 30%), while in few cases both-sided S-VHD were detected (7 patients, 7%). (C) When considering patients with multiple VHD (= more than one valve with severe dysfunction in the same patient), a total of 115 severe valvular dysfunctions were detected among the 101 patients with S-VHD. Severe regurgitant lesions were prevalent (75) as compared to severe stenotic lesions (37), while in three cases severe combined VHD was present (2 patients with severe pulmonary valve steno-insufficiency and 1 with severe mitral valve steno-insufficiency). (D) The pulmonary valve was the most frequently affected by severe stenosis and/or regurgitation (S-PVD, n = 41), followed by the tricuspid valve (S-TVD, n = 35). Severe aortic valve (S–AV) and mitral valve (S–MV) stenosis and/or regurgitation were less common (25 and 14 respectively).

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  FIGURE 3. Kaplan–Meier curves assessing the cumulative event-free survival rates in severe valvular heart disease (S-VHD) and NS-VHD populations. S-VHD, severe valvular heart disease; NS-VHD, non-severe valvular heart disease.

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  FIGURE 4. Echocardiographic images (Top: apical 4-chamber views, Bottom: CW Doppler through mitral valve and systemic AV valve) of four patients included in our study, coupled according to the ACHD [(A,B): repaired partial atrioventricular septal defect; (C,D): Fontan circulation], with (Panel B,D) and without (Panel A,C) associated S-VHD. This is illustrative of our findings, since patients (B–D) experienced hospitalization for heart failure. while patients (A–C) had an unremarkable clinical course.