Letters to the Editor

133

18p Monosomy Syndrome

Atrioventricular Block in the Neonate

We have a comment regarding the report of " H e a r t disease associated with deletion of the short arm of chromosome 18" [1]. We have followed from infancy to the present an 8-year-old girl who has 18p monosomy syndrome. This girl has many of the clinical features described: mental retardation, short stature, and abnormal facies which include severe clefts. She has dextrocardia with situs inversus. Doppler echocardiography and color flow mapping have demonstrated a moderate-sized malaligned subaortic ventricular septal defect (VSD) with mild pulmonary stenosis. We estimated a 20mm gradient across the right ventricular outflow tract. Her moderate-sized VSD is restrictive. There was a 45-mmHg pressure difference between the left and right ventricles with a moderate left-to-right shunt. She has remained free of problems related to her cardiovascular abnormality. Our patient presents yet another manifestation [2] of cardiac pathology with a 18p deletion syndrome. This study suggests that pediatricians of 18p monosomy patients may want to screen their patients accordingly.

In the article "Advanced atrioventricular block in a neonate with lupus erythematosus and anti-SS-A antibodies," [1], Fig. 2B was interpreted as a 3:1 block (during crying). I would like to point out that this interpretation is incorrect. In this figure, there are five pairs of QRS complexes separated by an interval of 0.64 s. Therefore, I think this figure should be interpreted as a 3 : 2 atrioventricular block, with two conducted and one nonconducted sinus P waves. The second and third sinus P waves are embedded in the preceding T waves. Bigeminy due to junctional premature beats is also not likely, because the first and second QRS complexes of each couple are identical.

References 1. Pearl W (1989) Heart disease associated with deletion of the short arm of chromosome 18. Pediatr Cardiol 10: 174-176 2. Bridge JA, McManus BM, Remmenga J, Cuppage FP (1989) Complete heart block in the 18p syndrome. Arch Pathol Lab Med 113:539-541

Henry A. Kane Digamber Borgaonkar Matthew McDermott Stuart Septimus Hamid Movahhedian Medical Center o f Delaware Delaware, USA

Reference 1. Shimizu T, Ino T, Nishimoto K, Iwahara M, Yamashiro Y, Yabuta K (1988) Advanced atrioventricular block in a neonate with lupus erythematosus and anti-SS-A antibodies. Pediatr Cardiol 9:122

Ming-Yi Wu Medical Department Shanghai Children's Hospital Shanghai, Peoples Republic" o f China

Atrioventricular block in the neonate.

Letters to the Editor 133 18p Monosomy Syndrome Atrioventricular Block in the Neonate We have a comment regarding the report of " H e a r t diseas...
57KB Sizes 0 Downloads 0 Views