Survival and Risk Factor Analysis for the Norwood Procedure for Hypoplastic Left Heart Syndrome

Kern, Jeffrey H.; Hayes, Constance J.; Michler, Robert E.; Gersony, Welton M.; Quaegebeur, Jan M.

doi:10.1016/s0002-9149(97)00313-5

Cited by 182 publications

(78 citation statements)

References 16 publications

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“…Several institutions have reported operative survival for the stage I procedure between 68% and 77%. [5][6][7] There has also been a reduction in operative mortality with subsequent staging procedures. 8,9 Modifications in the Fontan operation, such as fenestration of the Fontan baffle, and the introduction of intermediate staging with a bidirectional cavopulmonary anastomosis (BCPA) have contributed to this improved survival.…”

mentioning

confidence: 99%

Survival After Reconstructive Surgery for Hypoplastic Left Heart Syndrome : A 15-Year Experience From a Single Institution

Mahle

Spray²,

Wernovsky³

et al. 2000

Circulation

305

261

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Background-There are limited data regarding the long-term survival of patients who have undergone reconstructive surgery for hypoplastic left heart syndrome (HLHS). We reviewed the 15-year experience at our institution to examine survival in the context of continued improvements in early operative results. Methods and Results-Between 1984 and 1999, 840 patients underwent stage I surgery for HLHS. From review of medical records and direct patient contact, survival status was determined. The 1-, 2-, 5-, 10-, and 15-year survival for the entire cohort was 51%, 43%, 40%, 39%, and 39%, respectively. Late death occurred in 14 of the 291 patients discharged to home after the Fontan procedure, although only 1 patient has died beyond 5 years of age. Heart transplantation after stage I reconstruction was performed in 5 patients. Later era of stage I surgery was associated with significantly improved survival (PϽ0.001). Three-year survival for patients undergoing stage I reconstruction from 1995 to 1998 was 66% versus 28% for those patients undergoing surgery from 1984 to 1988. Age Ͼ14 days at stage I and weight Ͻ2.5 kg at stage I were also associated with higher mortality (Pϭ0.004 and Pϭ0.01, respectively). Other variables, including anatomic subtype, heterotaxia, and age at subsequent staging procedures, were not associated with survival. Conclusions-Over

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mentioning

confidence: 99%

Survival After Reconstructive Surgery for Hypoplastic Left Heart Syndrome : A 15-Year Experience From a Single Institution

Mahle

Spray²,

Wernovsky³

et al. 2000

Circulation

305

261

View full text Add to dashboard Cite

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“…[19][20][21] Neurologic complications have been noted to occur among infants with HLHS not only as a result of the surgical procedure but also during the preoperative and postoperative periods. [22][23][24]23,25 have documented seizures, cerebral palsy, attention-deficit disorder, and decreased IQ scores for children with HLHS who have undergone either palliative surgery or OHT. Although most morbidities encountered after surgical intervention are neurologic, renal failure, complete heart block, respiratory failure, and sepsis have also been documented.…”

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confidence: 99%

Clinical Outcomes and Secondary Diagnoses for Infants Born With Hypoplastic Left Heart Syndrome

et al. 2004

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ABSTRACT. Objective. To explore clinical outcomes and secondary diagnoses present at discharge for infants born with hypoplastic left heart syndrome (HLHS), from a national perspective.Methods. We examined hospitalizations for infants <30 days of age who were born with HLHS, using hospital discharge data from the 1997 Kids Inpatient Database. To explore treatment choices, clinical outcomes, and resource use, we used International Classification of Diseases, 9th Revision, Clinical Modification diagnostic and procedure codes to classify discharges according to type of surgical intervention versus no surgical intervention. To investigate outcomes in more detail, we identified secondary diagnoses noted at discharge, using International Classification of Diseases, 9th Revision, Clinical Modification codes, and stratified results according to type of surgical intervention.Results. Of a total of 550 patients with HLHS, 234 underwent the Norwood procedure, 17 underwent orthotopic heart transplantation, and 106 died in the hospital with no reported surgical intervention. Although we found no demographic variables to be significantly associated with the type of treatment received, discharged patients who died without surgical intervention were significantly more likely to have received care in hospitals identified as small (odds ratio [OR]: 1.5; 95% confidence interval [CI]: 1.03-3.1) or not children's hospitals (OR: 2.02; 95% CI: 1.13-3.6). Secondary diagnoses of cardiac arrest (OR: 2.0; 95% CI: 1.1-3.4) and seizures (OR: 2.6; 95% CI: 1.2-5.5) occurred more frequently in orthotopic heart transplantation cases than in Norwood procedure cases.Conclusions. These data from a national perspective reflect outcomes of infants with HLHS during a time when rates of initial survival after surgical intervention were considered to be improved. These findings may be useful to clinicians when they are considering and recommending initial medical and surgical strategies currently being proposed for the treatment of HLHS. H ypoplastic left heart syndrome (HLHS) remains one of the most complex congenital heart defects to treat, both medically and surgically. Despite advances in treatment, HLHS continues to have the highest mortality rate, of all congenital heart defects, for infants Ͻ1 year of age. 1 HLHS is a combination of congenital cardiac anomalies involving hypoplasia of the ascending aorta, aortic valve atresia or stenosis, a small or absent left ventricle, and mitral atresia or hypoplasia. 2 Before 1980, HLHS was considered universally fatal in the newborn period, leaving the health care team with the sole option of providing compassionate care. [2][3][4][5][6] Currently, most infants diagnosed as having HLHS undergo a series of surgical palliations; a small percentage undergo orthotopic heart transplantation (OHT) as an initial surgical approach. [7][8][9][10][11][12][13][14][15][16][17][18] As the rates of death after surgical intervention for HLHS have decreased, morbidities resulting from both surgical palliation and transpla...

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“…95% (40,45), por lo tanto, se ha postulado que este grupo de pacientes tendría un mayor beneficio al ser atendidos en un centro especializado al momento de nacer. De hecho la demora en el traslado al centro terciario fue identificada como uno de los factores de riesgo de muerte preoperatorio en este grupo (45) Un análisis retrospectivo comparó pacientes con transposición de grandes vasos; 68 recién nacidos con diagnóstico prenatal y 250 con diagnóstico postnatal.…”

Section: Diagnóstico Prenatal Y Pronóstico Neonatalunclassified

“…Sin embargo, el grupo de diagnóstico prenatal presentó menos eventos neurológicos adversos (15,2% v/s. 27,7%; OR; 0,43: 0,18-1,0) (43), y una mejor condición metabólica pre operatoria (40,42).…”

Section: Diagnóstico Prenatal Y Pronóstico Neonatalunclassified

El Diagnóstico Prenatal De Cardiopatías Congénitas Mejora El Pronóstico Neonatal

Quiroz

et al. 2006

Rev. chil. obstet. ginecol.

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RESUMENLas malformaciones congénitas son la principal causa de muerte neonatal precoz en nuestro medio; en la mayoría de los casos corresponden a cardiopatías congénitas mayores. Las cardiopatías congénitas tienen una incidencia de 8/1.000 recién nacidos vivos, correspondiendo la mitad de ellas a cardiopatías congénitas mayores. Una de las intervenciones recomendadas para reducir la mortalidad de este grupo de niños es evaluar rutinariamente la anatomía del corazón fetal mediante ecografía obstétrica, para planificar la atención neonatal, de aquellos fetos con cardiopatías congénitas, en el momento y lugar más oportuno. En objetivo de la presente revisión es comprobar si el diagnóstico prenatal de una cardiopatía congénita mejora el pronóstico perinatal respecto de aquellos casos que son diagnosticados post parto. Observamos que el diagnóstico antenatal de cardiopatía congénita, no mejora la sobrevida neonatal, excepto en ciertas cardiopatías congénitas ductus dependientes (transposición de grandes arterias, hipoplasia del corazón izquierdo y coartación de aorta), en que si se ha reportado una mayor probabilidad de sobrevida en el grupo de recién nacidos en los cuales se realizó el diagnóstico en el período prenatal. Recomendamos la evaluación rutinaria del corazón fetal en la ecografía obstétrica habitual, y efectuar ecocardiografía fetal especializada ante la sospecha de alteraciones o en aquellos grupos de mayor riesgo. PALABRAS CLAVES: Cardiopatías congénitas mayores, diagnóstico prenatal, ecocardiografía fetal SUMMARYCongenital malformations are the main cause of neonatal death; in most of the cases they correspond to major congenital heart defects. Congenital heart defects have an incidence of 8/1,000 live newborns, corresponding half of them to major congenital heart defects. To reduce the mortality of this group of children routine evaluation of fetal heart anatomy by ultrasound is recommended, allowing neonatal care, of those fetuses with congenital heart defects, at the appropriate time and place. Here we attempt to verify if prenatal diagnosis of a congenital heart defect improves the perinatal outcome compare to postnatal diagnosis. We observed that the prenatal diagnosis of congenital heart defects, does not improve the neonatal outcome, except in certain congenital ductus dependent heart defects (transposition of great vessels, hypoplastic left heart syndrome and aortic coarctation), where a better outcome has been shown for those new born with prenatal diagnosis. We recommend routine evaluation of the fetal heart during prenatal ultrasound, and to carry out fetal echocardiography in high risk groups or when a defect is suspected.

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Survival and Risk Factor Analysis for the Norwood Procedure for Hypoplastic Left Heart Syndrome

Cited by 182 publications

References 16 publications

Survival After Reconstructive Surgery for Hypoplastic Left Heart Syndrome : A 15-Year Experience From a Single Institution

Survival After Reconstructive Surgery for Hypoplastic Left Heart Syndrome : A 15-Year Experience From a Single Institution

Clinical Outcomes and Secondary Diagnoses for Infants Born With Hypoplastic Left Heart Syndrome

El Diagnóstico Prenatal De Cardiopatías Congénitas Mejora El Pronóstico Neonatal

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