Therapeutic approach and relapse in congenital aortic stenosis

Introduction: Congenital aortic stenosis results from abnormalities in the formation of the valve leaflets. These abnormalities include fusion of one or more valve leaflets, leading to bicuspid or unicuspid aortic valves. The incidence of bicuspid aortic valve is com-mon, however, only small percentage of such indivi-duals develop aortic stenosis during childhood years and is difficult to make a choice regarding therapy. This case calls into question the therapeutic decisional risks (the practice of valvuloplasty for children, which then requires valvular prosthesis or reintervention by comi-surotomy), but also the accelerated progression of the disease.

Methods: Descriptive Study. Analysis of clinical and paraclinical data (biological, ECG route, cardiac echo-graphy). It is presented the case of a 11 year old child diagnosed, at the age of 1, with bicuspid aortic valve and severe aortic stenosis. Progression went to tight aortic stenosis (V max Ao=6.9 ms, LV-Ao gradient: maximum=192mmHg, mean=96mHg), and NYHA III cardiac failure. At that point, because the patient beca-me symptomatic, with dyspnea at low efforts, surgery was necessary. The surgery could not be performed at optimal time due to the parents’ refusal. Surgery was performed at the age of 9 and consisted of ante-rior comisurotomy and resection of subaortic steno-sis. After surgery, a residual gradient persisted (LV-Ao gradient=36mmHg and V max Ao =3m /s), but with the improvement of the symptoms. The difficult part of the case appears at the time of re-evaluation, at a dis-tance of 2 years after the intervention, when the cardiac echography shows severe aortic restenosis, with high LV-Ao gradient values (LV-Ao=133mmHg, V max Ao ascd=5.7m/s). This case calls into question the therape-utic decisional risks (the practice of valvuloplasty for children, which then requires valvular prosthesis or reintervention by comisurotomy), but also the accele-rated progression of the disease.

Results: Aortic restenosis at 2 years after surgical inter-vention. Surgical reintervention indication (g LV-Ao= 133mmHg, V max Ao ascd=5.7m /s).

Conclusions: The presented case shows the difficulty in deciding the surgical therapeutic technique (valvu-lotomy / valvuloplasty with known risks), the accelera-ted progression of the restenosis and the importance of postoperative cardiologic surveillance in pediatric patients with severe valvular pathology.

ISSN – online: 2734 – 6382
ISSN-L 1220-658X
ISSN – print: 1220-658X
The Romanian Journal of Cardiology is indexed by:
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CODE: 379
CME Credits: 10 (Romanian College of Physicians)