Natural history of Ebstein's anomaly of tricuspid valve in childhood and adolescence. An international co-operative study of 505 cases.

Abstract

505 cases of Ebstein's anomaly have been collected from 6i centres in 28 countries: 258 were male and 247 were female. Thirty-five (infants) were less than I year old, 403 (children and adolescents) were between i and 25years old, and 67 (adults) were over 25years old. Of the infants, 72 per cent were in heart failure, but in 8I per cent of the others growth and development during infancy was said to have been average or good. Of the children and adolescents, 7I per cent, and of the adults, 6o per cent, had little or no disability and were classified as cardiac grade I or IL Catheterizations were carried out on 363patients, with I3 deaths and 6 cardiac arrests: paroxysmal tachycardia occurred during the procedure in 9o of them. Necropsy was carried out in 93 cases: 48 per cent of those catheterized and 8i per cent of those coming to necropsy had associated congenital cardiac malformations. If defects in the interatrial septum are excluded the figures are I2 per cent and 42 per cent, respectively. The presence of an additional lesion appeared to increase the risk in those having surgical treatment.Of the series, IO per cent had ventricular pre-excitation and 28 per cent gave a history of paroxysmal dysrhythmia. Their presence was predictive for neither death nor dysrhythmia during cardiac catheterization, nor for sudden natural death.Of the 505, 77 (I3'3%) diedfrom natural causes, and 3I (54.4%) of the 57 who had surgical treatment did not survive operation. Analysis of the surgical experience suggests that palliative procedures have little or nothing to offer and that replacement of the malformed tricuspid valve should be delayed, ifpossible, until the patient's heart is large enough to take an adult-size prosthesis or homograft.on July 15, 2020 by guest. Protected by copyright.

Impact of Surgery in Ebstein’s Anomaly Using Current Surgical Criteria

“…10,16, 17 We also found that low LVEF was associated with lower survival. Alterations in LV geometry cause changes in LV systolic function, 18 and impaired LV systolic function is an independent predictor of mortality in EA (OR, 4.10; P=0.002), 19 this is further supported by the present study.…”

Ebstein’s Anomaly

“…58 The initial publication on patients with Ebstein's anomaly undergoing tricuspid valve replacement reported a surgical mortality of 54%. 49 Similar high early mortality and unsatisfactory late results for tricuspid valve repair by the methods available at that time were described. 57,59 Between April 1972 and January 2005, 540 consecutive patients with Ebstein's anomaly were operated on at Mayo Clinic Rochester.…”

“…In addition, 71% of children and adolescents and 60% of adults were classified as New York Heart Association functional class I or II. 49 A high mortality rate from congestive heart failure was noted during the first few months of life; subsequently, mortality plateaued at an average of 12% scattered uniformly throughout childhood and adolescence. Of those who had surgical treatment, 54% did not survive the operation.…”

“…15,16,48 The downward displacement of the septal leaflet of the tricuspid valve is associated with discontinuity of the central fibrous body and septal atrioventricular ring with direct muscular connections, thus creating a potential substrate for accessory atrioventricular connections and pre-excitation. 5,6 From 6% to 36% of patients with Ebstein's anomaly have Ն1 accessory pathways, 26,38,46,49,50 and most accessory pathways are located around the orifice of the malformed tricuspid valve. 25,27,47 Correct identification and treatment of accessory pathways are essential and may help to prevent sudden cardiac death.…”

“…49,76 -78 The largest of these studies, reporting on 505 patients with Ebstein's anomaly, was published Ͼ30 years ago. 49 The study consisted primarily of patients between 1 and 25 years of age, with 67 patients Ͼ25 years and only 35 patients Ͻ1 year of age. Of the infants Ͻ1 year of age, 72% were in heart failure, but for 81% of the others, growth and development during infancy were average or good.…”