Article

Perinatal and early surgical outcome for the fetus with hypoplastic left heart syndrome: a 5-year single institutional experience

Fetal Heart Program, The Cardiac Center, The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.
Ultrasound in Obstetrics and Gynecology (Impact Factor: 3.56). 10/2010; 36(4):465-70. DOI: 10.1002/uog.7674
Source: PubMed

ABSTRACT To review our experience with the prenatal diagnosis of hypoplastic left heart syndrome (HLHS). Our goal was to establish the benchmark for perinatal and early surgical outcome in the current era, from a center with an aggressive surgical approach and a cohort with a high level of intention-to-treat.
Outcome was assessed in fetuses with HLHS following stratification into high-risk and standard-risk categories. High risk was defined as the presence of any of the following: extracardiac, genetic or chromosomal anomalies; prematurity of < 34 weeks' gestation; additional cardiac findings such as intact or highly restrictive atrial septum, severe degree of tricuspid regurgitation or severe ventricular dysfunction. Standard risk was defined as absence of these risk factors.
Of 240 fetuses evaluated over 5 years, 162 (67.5%) were in the standard-risk group and 78 (32.5%) were in the high-risk group. Of the 240 sets of parents, 38 (15.8%) chose termination or non-intervention at birth at initial prenatal counseling and 185 of the neonates (77.1%) underwent first-stage Norwood surgery with 155 surviving and 30 deaths, giving an overall Norwood operative survival of 83.8%. Breakdown by risk class reveals a significant Norwood operative survival advantage for the standard-risk group (92.8%) over the high-risk group (56.5%) (P < 0.001).
Following prenatal diagnosis of HLHS, families should be strongly encouraged to undertake comprehensive prenatal evaluation in order to obtain an accurate prognosis. One-third have additional risk factors that limit survival outcome, however two-thirds do not and have an excellent chance of early survival.

0 Bookmarks
 · 
102 Views
  • [Show abstract] [Hide abstract]
    ABSTRACT: Hypoplastic left heart syndrome (HLHS) is one of the most challenging forms of congenital heart disease to treat. Management of this condition consumes a substantial amount of energy, time, and bed capacity in many congenital heart centers in the western world today. Simply defined, HLHS is a congenital malformation in which the left ventricle is inadequate and non-viable in its capacity to perform the function of systemic perfusion. The current conventional strategy of staged reconstruction involves the principles of right ventricular recruitment for systemic perfusion, aortic arch reconstruction and a two-step process of channeling systemic venous return directly to the pulmonary vasculature absent the use of a ventricular pump (bidirectional Glenn and Fontan operations). Initially promoted by Norwood in the early 1980's, the management protocol continues to evolve, leading to the survival of thousands of children who would otherwise succumb to this lethal condition.
    Circulation 07/2014; 130(8). DOI:10.1161/CIRCULATIONAHA.114.011728 · 14.95 Impact Factor
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Presented is a retrospective outcome study of a 15-year single institutional experience with a contemporary cohort of patients with hypoplastic left heart syndrome and complex that underwent a "Giessen Hybrid" stage I as initial palliation. Hybrid approach consisting of surgical bilateral pulmonary artery banding and percutaneous duct stenting with or without atrial septum manipulation was developed from a rescue approach to a first-line procedure. Comprehensive Aristotle score defined pre-operative condition. Fifteen-year follow-up mortality is reported as occurring within the staged univentricular palliation or before and after biventricular repair. Hybrid stage I was performed in 154 patients; 107 should be treated by single ventricle palliation, 33 by biventricular repair (BVR), 7 received heart transplantation, and 7 were treated by comfort care, respectively. Overall 34 children died. The Aristotle score (mean value 18.2 ± 3) classified for univentricular circulations in newborns did not have statistical impact on the outcome. Two patients died during stage I (1.2 %), and the interstage I mortality was 6.7 %, and stage II mortality 9 %, respectively. Stage III was up to now performed in 57 patients without mortality. At 1 year, the overall unadjusted survival of HLHS and variants was 84 % and following BVR 89 %, respectively. The Fifteen-year survival rate for HLHS and variants was 77 %, with no significant impact of birth weight of less than 2.5 kg. In conclusion, Hybrid stage I fulfilled the criteria of life-saving approach. In our institution, Hybrid procedure replaced Norwood-staged palliation with a considerable mid- and long-term survival rate. Considering interstage mortality close surveillance is mandatory.
    Pediatric Cardiology 09/2014; 36(2). DOI:10.1007/s00246-014-1015-2 · 1.55 Impact Factor
  • [Show abstract] [Hide abstract]
    ABSTRACT: Within the last few decades the pre-natal echocardiographic diagnosis of congenital heart defects has made substantial progresses, particularly for the identification of complex malformation. "Functionally" univentricular hearts categorize a huge variety of heart malformations. Since no one of the patients with these congenital heart defects can ever undergo a bi-ventricular type of repair, early recognition and decision-making from the neonatal period are required in order to allow for appropriate multiple-step diagnostic and treatment procedures, either of interventional cardiology and/or surgery, on the pathway of "univentricular" heart. In the literature strong disagreements exist about the potential impact of the pre-natal diagnosis on the early and late outcomes of complex congenital heart defects. This review of the recent reports has been undertaken to better understand the impact of pre-natal diagnosis in "functionally" univentricular hearts taking into consideration the following topics: pre-natal screening, outcomes and survival, general morbidity, neurologic and developmental consequences, pregnancy management and delivery planning, resources utilization and costs/benefits issues, ethical implications, parents counseling, and interruption of pregnancy versus treatment.
    Frontiers in Pediatrics 01/2015; 3:15. DOI:10.3389/fped.2015.00015

Preview

Download
1 Download