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: 3.85). 10/2010; 36(4):465-70. DOI: 10.1002/uog.7674
Source: PubMed


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.

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    • "However, tricuspid regurgitation (TR) remains an important risk factor that affects their survival, and there have been many reports regarding the negative impact of TR on the clinical course of HLHS [1] [2] [3] [4] [5] [6] [7] [8]. Some patients show significant TR at birth, which may be diagnosed prenatally by fetal echocardiography [9] [10], and tend to present a difficult clinical course. Others show a gradual increase in TR as they grow. "
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    ABSTRACT: Tricuspid regurgitation (TR) remains a significant risk factor affecting the survival of patients with hypoplastic left heart syndrome (HLHS). We performed this study to investigate differences in the clinical course based on the timing of the development of TR and the effects of tricuspid valve surgery (TVS). One hundred and five patients of classic HLHS underwent staged operations from May 1991 to July 2010. Forty-four patients (41.9%) exhibited moderate or greater TR during the follow-up. We defined the early TR group (30 patients, around the first palliative surgery) and the late TR group (14 patients, the later period) based on the timing of the appearance of moderate or greater TR. We performed TVS when moderate or greater TR was detected in 28 patients. The follow-up period was 5.5 ± 5.1 (plus/minus values are means ± SD) years (range: 0.01-14.6 years) after the first palliative surgery and 4.9 ± 4.4 years (range: 0.01-13.3 years) after TVS. The early TR group exhibited poorer survival than the late TR group (42.9 vs 92.9% at 5 years, P = 0.003). However, in the early TR group, the TVS significantly improved survival compared with that observed in the non-TVS cases (52.1 vs 23.3% at 5 years, P = 0.046). The right ventricular ejection fraction (RVEF) significantly decreased (62.7 ± 11.4→57.2 ± 12.6% (plus/minus values are means ± SD), P = 0.040) and the right ventricular end-diastolic diameter (RVDd) became significantly enlarged (27.7 ± 7.6→36.7 ± 3.4 mm, P < 0.001) in association with deterioration of the TR degree. TVS significantly improved the degree of TR (2.5 ± 0.5→1.5 ± 0.9°, P < 0.001) and RVDd (37.7 ± 7.4→30.4 ± 5.0 mm, P = 0.007); however, the RVEF was not improved 1 month after surgery (54.4 ± 12.1→54.3 ± 12.4%, P = 0.931) or at the latest follow-up (53.7 ± 14.9%, P = 0.836). The survival of HLHS patients who develop moderate or greater TR around the time of the first palliative surgery is worse than that of HLHS patients who develop moderate or greater TR at a later time. In this study, TVS for early TR improved survival and decreased right ventricular dimensions during the 4.9-year follow-up period.
    Full-text · Article · Jan 2014 · European journal of cardio-thoracic surgery: official journal of the European Association for Cardio-thoracic Surgery
    • "Prenatal diagnosis of HLHS has several specific advantages that include improved counseling, directed and planned deliveries at specialized centers, and reduced perioperative morbidity.[1819] These advantages may be even greater in emerging economies where neonatal transport is poorly developed. "
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    ABSTRACT: The evolution of surgical skills and advances in pediatric cardiac intensive care has resulted in Norwood procedure being increasingly performed in emerging economies. We reviewed the feasibility and logistics of performing stage one Norwood operation in a limited-resource environment based on a retrospective analysis of patients who underwent this procedure in our institution. Retrospective review of medical records of seven neonates who underwent Norwood procedure at our institute from October 2010 to August 2012. The median age at surgery was 9 days (range 5-16 days). All cases were done under deep hypothermic cardiopulmonary bypass and selective antegrade cerebral perfusion. The median cardiopulmonary bypass (CPB) time was 240 min (range 193-439 min) and aortic cross-clamp time was 130 min (range 99-159 min). A modified Blalock-Taussig (BT) shunt was used to provide pulmonary blood flow in all cases. There were two deaths, one in the early postoperative period. The median duration of mechanical ventilation was 117 h (range 71-243 h) and the median intensive care unit (ICU) stay was 12 days (range 5-16 days). Median hospital stay was 30.5 days (range 10-36 days). Blood stream sepsis was reported in four patients. Two patients had preoperative sepsis. One patient required laparotomy for intestinal obstruction. Stage one Norwood is feasible in a limited-resource environment if supported by a dedicated postoperative intensive care and protocolized nursing management. Preoperative optimization and prevention of infections are major challenges in addition to preventing early circulatory collapse.
    No preview · Article · Mar 2013 · Annals of Pediatric Cardiology
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    • "Thus, the incidence of neonatal reparative operations is increasing (Backer & Mavroudis, 2007). With the advancement of surgical techniques and perioperative care, survival rates for all congenital heart defects are improving, including the most lethal defect hypoplastic left heart syndrome (HLHS) (Rychik, 2010). Given the advances in pre-natal diagnosis and medical/surgical management practices, CHD is quickly becoming the most common chronic illness in childhood (Tak & McCubbin, 2002). "
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    ABSTRACT: To ascertain the primary caregiver's postdischarge perceptions of infant care issues after neonatal heart surgery. Fifteen primary caregivers of infants who had neonatal heart surgery for complex congenital heart disease (CHD) participated in this study. We conducted two focus groups and four individual phone interviews using a structured interview guide. The topics included parent feeding management, infant caloric intake, parental acceptance of nasogastric tube, infant feeding behaviors, and issues of parenting stress. We audio recorded focus group sessions, made detailed notes and key quotes were recorded verbatim by a certified impartial focus group facilitator. Feeding problems were present in both infants who were on full oral feeds and infants who were dependent on supplemental feeding tubes. Mothers of infants with feeding problems expressed concern over infant weight gain and caregiver sleep deprivation, which largely contributed to parental stress. In this small study of infants who experienced neonatal surgery for complex CHD, parental stress over feeding and weight gain were identified as important areas to be addressed during hospitalization. Future studies are needed to address increased at-home parental support.
    Full-text · Article · Mar 2012 · MCN. The American journal of maternal child nursing
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