ATRIOVENTRICULAR VALVE DYSPLASIA IN 22 NEWBORN-INFANTS

We retrospectively studied the experience of our institution with isol ated dysplasia of one or both atrio-ventricular valves in 22 newborn i nfants. All patients with associated cardiac malformations were exclud ed. Ten patients exhibited isolated tricuspid valve dysplasia. One pat ient had tricuspid valve dysplasia and a dysplastic pulmonary valve. I n 10 patients, both atrio-ventricular valves were affected. Finally, m itral valve dysplasia was associated with pulmonary valve stenosis in 1 case. Associated syndromes and/or chromosomal anomalies were: Down s yndrome (n=2), trisomy 18 (n=1), Noonan syndrome (n=1), Marfan syndrom e (n=3), Ehlers-Danlos and Cutis laxa (n=2). Mortality was 27.2% durin g follow-up (mean 51 months): 3 patients with chromosomal aneuploidies , 2 patients with severe neonatal Marfan syndrome and 1 with Ehlers-Da nlos. Complications were: sustained supra-ventricular tachycardia in 3 , neonatal staphylococcal tricuspid valve endocarditis in 1, persisten t significant valvular disease in 8. In the remaining 9 survivors, the dysplasia of the atrio-ventricular valves persists with absent or mil d incompetence. Beside obvious chromosomal anomalies, newborn infants with dysplastic valves should be investigated for manifestations of co nnective tissue disorders. This may help to identify new pleiotropic s yndromes which include valvular dysplasia as one manifestation. (C) 19 97 Elsevier Science Ireland Ltd.