| Title |
Perinatal Outcome in Fetuses with Heterotaxy Syndrome and Atrioventricular Block or Bradycardia
|
|---|---|
| Published in |
Pediatric Cardiology, February 2014
|
| DOI | 10.1007/s00246-014-0874-x |
| Pubmed ID | |
| Authors |
Maria C. Escobar-Diaz, Wayne Tworetzky, Kevin Friedman, Terra Lafranchi, Francis Fynn-Thompson, Mark E. Alexander, Douglas Y. Mah |
| Abstract |
Congenital atrioventricular (AV) block is commonly associated with heterotaxy syndrome; together they have reportedly low survival rates (10-25 %). However, information about perinatal outcome and predictors of non-survival after prenatal diagnosis of this association is scarce. Therefore, we studied fetuses with heterotaxy syndrome and bradycardia or AV-block diagnosed between 1995 and 2011, and analyzed pre and post-natal variables. The primary outcome was death and the secondary outcome was pacemaker placement. Of the 154 fetuses with heterotaxy syndrome, 91 had polysplenia syndrome, 22/91(24 %) with bradycardia or AV-block. Thirteen (59 %) patients had sinus bradycardia at diagnosis, 8 (36 %) complete AV block, and 1 (5 %) second-degree AV-block. Three patients elected for termination of pregnancy (3/22, 14 %), 4 had spontaneous fetal demise (4/22, 18 %), and 15 (15/22, 68 %) were live-born. Of the fetuses with bradycardia/AV-block, 30 % presented with hydrops, 20 % had ventricular rates <55 beats/min, and 10 % had cardiac dysfunction. Excluding termination of pregnancy, 15/19 fetuses (79 %) survived to birth. Among the 15 live-born patients, 4 had bradycardia and 11 had AV-block. A further 3 patients died in infancy, all with AV-block who required pacemakers in the neonatal period. Thus, the 1-year survival rate, excluding termination of pregnancy, was 63 % (12/19). Of the remaining 12 patients, 9 required pacemaker. Predictors of perinatal death included hydrops (p < 0.0001), ventricular dysfunction (p = 0.002), prematurity (p = 0.04), and low ventricular rates (p = 0.04). In conclusion, we found a higher survival rate (63 %) than previously published in patients with heterotaxy syndrome and AV block or bradycardia diagnosed prenatally. Hydrops, cardiac dysfunction, prematurity and low ventricular rates were predictors of death. |
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Geographical breakdown
| Country | Count | As % |
|---|---|---|
| Unknown | 2 | 100% |
Demographic breakdown
| Type | Count | As % |
|---|---|---|
| Members of the public | 2 | 100% |
Mendeley readers
The data shown below were compiled from readership statistics for 44 Mendeley readers of this research output. Click here to see the associated Mendeley record.
Geographical breakdown
| Country | Count | As % |
|---|---|---|
| Korea, Republic of | 1 | 2% |
| South Africa | 1 | 2% |
| Unknown | 42 | 95% |
Demographic breakdown
| Readers by professional status | Count | As % |
|---|---|---|
| Student > Ph. D. Student | 7 | 16% |
| Professor > Associate Professor | 6 | 14% |
| Other | 5 | 11% |
| Researcher | 5 | 11% |
| Student > Bachelor | 4 | 9% |
| Other | 10 | 23% |
| Unknown | 7 | 16% |
| Readers by discipline | Count | As % |
|---|---|---|
| Medicine and Dentistry | 27 | 61% |
| Nursing and Health Professions | 3 | 7% |
| Arts and Humanities | 1 | 2% |
| Economics, Econometrics and Finance | 1 | 2% |
| Agricultural and Biological Sciences | 1 | 2% |
| Other | 0 | 0% |
| Unknown | 11 | 25% |
Attention Score in Context
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#15,298,293
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Outputs from Pediatric Cardiology
#649
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#191,201
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Outputs of similar age from Pediatric Cardiology
#29
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