Subject(s)
Heart Conduction System/physiopathology , Lown-Ganong-Levine Syndrome/diagnosis , Wolff-Parkinson-White Syndrome/diagnosis , Adolescent , Adult , Diagnosis, Differential , Female , Heart Function Tests , Heart Ventricles/innervation , Humans , Lown-Ganong-Levine Syndrome/physiopathology , Male , Middle Aged , Wolff-Parkinson-White Syndrome/physiopathologyABSTRACT
Medical genetic consultation was provided for 35 patients with Wolff-Parkinson-White syndrome (WPWS). The investigators ascertained the family history, inquired and examined patients, referred the patients' relatives for ECG and echocardiographic investigations. A newly diagnosed WPWS was reported in 3, Clerc-Levy-Cristesco (CLC) syndrome in 17, CLC phenomenon in 45 out of 132 grade I and II relatives of the proband. The study confirmed autosomal dominant inheritance of WPWS. More frequent occurrence of the syndrome was noted in the proband's relatives in case the disease occurred in both parents, less frequent in families with the history of one WPWS-affected parent. It is suggested that the disease should be considered associated with hereditary predisposition.
Subject(s)
Wolff-Parkinson-White Syndrome/genetics , Adolescent , Adult , Aged , Aged, 80 and over , Child , Child, Preschool , Echocardiography , Electrocardiography , Female , Humans , Male , Middle Aged , Pedigree , Wolff-Parkinson-White Syndrome/diagnosisABSTRACT
Primary prolapse of the mitral valve was diagnosed in 27 (11.7%) out of 230 patients with preexcitation syndrome: 10 of them presented with Wolff-Parkinson-White syndrome, 15 with Clerc-Lévy-Critesco syndrome, and two patients with combination of both syndromes. Preexcitation syndrome combined with mitral valve prolapse manifested itself by different disorders of the cardiac rhythm, mainly by the supraventricular forms: by PST in 17, nonparoxysmal tachycardia in 1, atrial fibrillation paroxysms in 7, sinus tachycardia in 2, supraventricular extrasystole in 3, pacemaker migration in the atria in 1 patient. Clerc-Lévy-Critesco syndrome initiated with atrial fibrillation paroxysms in 7 patients with mitral valve prolapse and with the enlargement of the left atrium. The prognostically unfavourable varieties of the preexcitation syndrome and mitral valve prolapse manifested themselves in ventricular tachycardia progressing to ventricular fibrillation, paroxysmal supraventricular tachycardia with a high frequency of ventricular contractions up to 213-230 and atrial tachyarrhythmia up to 250-280 per minute, which were regarded as factor at risk for potential transformation to ventricular tachycardia; as well as in continuously recurrent attacks of arrhythmia in the presence of multiple AV additional tracts or combined with sinoatrial and atrioventricular block.