Outcome after prenatal diagnosis of the hypoplastic left heart syndrome

Abstract

Objective-To derive accurate survival figures in the current surgical era for counselling in early pregnancy after the diagnosis of fetal hypoplastic left heart syndrome. Setting-A tertiary referral centre for paediatric cardiology and cardiac surgery. Design-A retrospective study of the outcome in all cases of hypoplastic left heart syndrome presenting in fetal life between mid-1993 and the end of 1996. Patients-The diagnosis was made in 30 fetuses. In four of 12 identified before 24 weeks' gestation the mothers chose to terminate the pregnancy. There was an intention to treat in 24 of the remaining fetuses. Main outcome measure-Survival to six months of postnatal life. Results-Of the 24 infants, five were not oVered Norwood stage 1 because of trisomy 18 (n = 2), unfavourable cardiac anatomy (n = 2), or neurological impairment (n = 1). One further infant did not survive to cardiac surgery after gastrointestinal surgery. Of the remaining 18 patients, eight had features that were considered to increase the risk of surgical repair. Of the 18 patients who underwent Norwood stage 1, there were nine survivors. There was a survival rate of 70% in infants undergoing surgery with no complicating features, a 50% survival of the all surgical candidates, and 37.5% survival from an intention to treat position. Conclusions-At the initial diagnosis of fetal hypoplastic left heart syndrome, the overall survival appears to be less than 40%. Evaluation must include detailed extracardiac and intracardiac assessment to predict the risks of surgical treatment. Prenatal counselling can be modified as pregnancy advances, depending on the detection or exclusion of complicating factors.

Accuracy of the Fetal Echocardiography in the High Risk Pregnancies

“…27,57 Improvements from the point of view of the public health could be achieved in better screening protocols, broadly performed by more skilled professionals in earlier gestational ages. [58][59][60] Increasing role of 3D or live 3D ultrasonography, and telemedicine are promising fields of the development of fetal echocardiography, with the possibility to improve detection rate and accuracy of prenatal CHD diagnosis. [9][10][11][61][62][63][64][65][66] Goal of the development of fetal echocardiography should be to increase prenatal detection rate of most hemodynamically significant CHD in early pregnancy in order to decrease late diagnosis of CHD postnatally.…”

El Diagnóstico Prenatal De Cardiopatías Congénitas Mejora El Pronóstico Neonatal

“…Estos problemas asociados explican por ejemplo la gran variabilidad reportada en la sobrevida neonatal, con tasas entre el 38 y 77% (29)(30)(31)(32). Otra fuente de confusión es la interrupción electiva del embarazo en algunos países donde el aborto es legal y culturalmente aceptable, dado el mal pronóstico perinatal (33).…”

“…Sin embargo, el grupo de pacientes a quienes se realizó el diagnóstico prenatalmente presentaron con menor frecuencia acidosis metabólica (6% v/s 45%) y menos episodios de paro cardiaco (0% v/s 9%) (32)(33)(34)(35).…”

“…La mortalidad postoperatoria es de un 40-50% aproximadamente (31,38) y los trastornos neurocognitivos son frecuentes. El parto en un centro neonatal especializado permitiría la administración precoz de prostaglandinas reduciendo el riesgo de hipoxia y muerte (33).…”

Interventional fetal balloon valvuloplasty for congenital heart disease—current shortcomings and possible perspectives

“…Despite recent advances, prenatal detection and accuracy of CHD diagnosis still remain problematic; this situation is compounded by the difficulties of assessing the adequacy of ventricular size and function with a view to future suitability for biventricular or univentricular repair in some forms of CHD [43][44][45][46]. Some cardiac lesions may evolve during pregnancy, so clarification of the implications of fetal cardiac findings may need to be delayed until later in gestation [12,25,26,32,33,[43][44][45][46][47].…”