Initial Counseling Prior to Palliation for Hypoplastic Left Heart Syndrome

Objective. Multiple surgical approaches to the initial palliation of patients with hypoplastic left heart syndrome (HLHS) have been advocated throughout the years. We sought to examine what procedure, if any, is recommended for HLHS management in regard to physician preference, anatomical variations, and concomitant medical issues. Patients and Methods. A Web‐based survey of pediatric cardiologist subscribed to PediHeart was conducted. Outcome Measures. The types of palliation recommended (Norwood palliation, Sano modification, hybrid palliation, primary cardiac transplantation, or hospice care) for patients with HLHS with anatomic or comorbid variants were queried. Counseling provided by the physicians to families was also documented as regards survival and outcomes. Results. Two hundred physicians (21% female) who averaged 12.3 years removed from training responded to the survey. US East Coast and Midwest respondents were more likely to recommend Norwood palliation (54% and 60%, respectively) and the US South and West respondents preferred Sano modification (73% and 82%, respectively). Norwood or Sano palliation was recommended over hospice care, hybrid palliation, or cardiac transplant for patients with an intact atrial septum (P < 0.05), moderate to severe tricuspid regurgitation (P < 0.05), or low birth weight defined as less than 2 kg (P < 0.05). Hospice was preferred in low‐birth‐weight infants over hybrid palliation or cardiac transplantation (P < 0.05). Hospice was recommended over any other palliation for premature infants (less than 30 weeks gestation), chromosomal abnormalities, or end‐organ dysfunction (P < 0.05). Conclusions. This survey demonstrates that different palliative options are primarily recommended by caretakers based upon institutional location and patient characteristics. Prospective comparative trials may force a rethinking of this approach over time.     

Impact of Staged Palliation on Somatic Growth in Patients with Hypoplastic Left Heart Syndrome

Background. Somatic growth pattern in infants undergoing staged palliation for hypoplastic left heart syndrome (Norwood procedure [NP], stage 2 palliation [S2P], and Fontan procedure [FP]) during transition toward a more energy efficient series circulation is not well understood. Objectives. We sought to determine growth pattern in these infants and factors influencing it. Methods. Patients who underwent NP since 2001 and survived at least 1 year after S2P were followed until FP or death/heart transplantation (n = 46). Weight for age z-scores (WAZ) were measured prior to NP; at initial discharge; prior to S2P; at four time periods after S2P; and prior to FP. Impact of gender, race, era of NP, anatomic subtype, NP shunt type, gastrostomy, home-surveillance program (daily weights and pulse oximetry), and interstage oxygen saturation on WAZ was evaluated. Results. Repeated measures anova showed a significant change in WAZ across time periods (P < 0.0005), with a significant decline from Pre-NP to Pre-S2P (P < 0.0005) and steady increase from Pre-S2P to Pre-FP (P= 0.016). None of the factors evaluated had a significant effect on this growth pattern. Length of hospital stay after NP was negatively correlated with WAZ at discharge (P= 0.001), but not for other time periods. Intervals from discharge to S2P and NP to S2P interval did not correlate with WAZ. Conclusion. Somatic growth is significantly impaired after NP but recovers steadily following S2P, as the patient transitions toward separated series circulation. Earlier S2P may lead to earlier resumption of normal growth in this critical period of early infancy.     

Successful Pregnancies in Two Women with Hypoplastic Left Heart Syndrome

Objective. Hypoplastic left heart syndrome (HLHS) is a relatively common complex congenital heart defect. Prior to development of staged reconstruction (i.e., Norwood procedure), HLHS was almost universally fatal within months of birth. Early survivors of the Norwood procedure are now reaching reproductive age. We report successful pregnancies in two such women. Patients. The first patient was a 20-year-old woman transferred from a community hospital at 33 3/7 weeks gestation because of preterm labor, suspected preeclampsia, and mild chronic hypoxemia. She had normal systemic ventricular shortening without significant valvar regurgitation but severe neoaortic dilatation. A fetal ultrasound demonstrated intrauterine growth restriction. An urgent Cesarean section was performed at 33 6/7 weeks gestation, given breech position and intractable preterm labor. The second patient, a 23-year-old woman followed at this institution through pregnancy, presented with preterm labor at 36 weeks gestation. Her systemic ventricular shortening was normal, with mild tricuspid regurgitation but without neoaortic dilation or regurgitation. She developed active labor at 36 3/6 weeks, and had a spontaneous vaginal delivery of a small for gestational age infant. Both women tolerated labor and childbirth without complication. Neither infant had evidence of structural heart disease on fetal echocardiography or physical examination. Conclusions. These cases, the first reported successful pregnancies in mothers with HLHS, highlight the challenges of pregnancy among women with complex congenital heart disease in general and raise several considerations specific to HLHS.     

Thromboses in the Native Aorta in Patients with Hypoplastic Left Heart Syndrome

We present four children with hypoplastic left heart syndrome (HLHS) and development of thrombus in the native aortic root. There are only two previously reported cases in the literature. In all four of our patients, thrombus was noted on transthoracic echocardiography. In one patient, surgical thrombectomy was performed, with subsequent deterioration and death. Thrombolysis was used in one patient, with suspected embolic events following his treatment. In the remaining two patients, medical management with chronic anticoagulation was successful in reducing the size of the thrombus.     

Palliation via Hybrid Procedure of a 1.4‐kg Patient with a Hypoplastic Left Heart

Despite improvements in survival of patients with hypoplastic left heart syndrome (HLHS) with various palliative procedures, certain risk factors, such as weight less than 2.5 kg, continue to predict increased mortality. We report the palliation of a patient with HLHS weighing 1.4 kg via a hybrid procedure consisting of banding of the pulmonary arteries bilaterally, stenting the ductus arteriosus, and balloon atrial septostomy. We propose that this may be another alternative for palliation in this high‐risk patient group.     

Hypoplastic Left Heart Syndrome in US Military Family Members: Trends in Intervention,Survival, and Prevalence

Introduction. Hypoplastic left heart syndrome was uniformly fatal until the 1980s but now has survival approaching 70% to age 5 due to treatment advances. The current study was undertaken to examine trends in the intervention rate, survival, and prevalence of hypoplastic left heart syndrome among US military family members. We hypothesized that increased rates of intervention and survival would produce a demonstrable increase in prevalence in this population. Methods. This was a retrospective review of administrative data. Central databases contain records from Army hospital admissions since 1972, all military hospital admissions since 1989, outpatient visits since 2000, and civilian bills to the military system since 2000. Patients with an instance of the relevant diagnosis (code 746.7) were included and all of their data records reviewed. Patients with incompatible diagnoses or survival were excluded. Results. There were 366 patients identified, 63 born in the 1980s, 155 in the 1990s, and 148 after 2000. The rate of intervention increased from 54% in the 1990s to 97% in the 2000s (P < .001). Five-year survival increased markedly between the 1980s and the 1990s. For patients born since 2000 and undergoing intervention, survival was 66% to 21 months (95% confidence interval 56–75%). Prevalence at birth in military hospitals was 1.4 per 10 000 with no significant trend over time. Prevalence among inpatients other than at birth increased from 0.4 to 15 per 10 000 between 1989 and 2005 (r² = 0.92, P < .001). Conclusions. The rates of intervention and survival of patients with hypoplastic left heart syndrome have increased dramatically over the past 15 years. This has resulted in a demonstrable increase in prevalence among the beneficiary population. Future study focused on this cohort could detail their healthcare needs and demonstrate the effect of hypoplastic left heart syndrome on the family and its growing impact on the military healthcare system.     

Balloon Atrial Septostomy by a Right Internal Jugular Venous Approach in a Newborn with Hypoplastic Left Heart Syndrome with a Restrictive Atrial Septum

Hypoplastic left heart syndrome with an intact or highly restrictive atrial septum requires urgent decompression of the left atrium. Catheter‐based interventions from the femoral or umbilical veins represent the standard method of atrial decompression. Restrictive atrial septal defects located at the superior portion of the fossa ovalis can be difficult to cross from these access sites. Here, we describe a successful Rashkind balloon atrial septostomy performed from an internal jugular approach.     

Nutrition Algorithms for Infants with Hypoplastic Left Heart Syndrome; Birth through the First Interstage Period

Failure to thrive is common in infants with hypoplastic left heart syndrome and its variants and those with poor growth may be at risk for worse surgical and neurodevelopmental outcomes. The etiology of growth failure in this population is multifactorial and complex, but may be impacted by nutritional intervention. There are no consensus guidelines outlining best practices for nutritional monitoring and intervention in this group of infants. The Feeding Work Group of the National Pediatric Cardiology Quality Improvement Collaborative performed a literature review and assessment of best nutrition practices from centers participating in the collaborative in order to provide nutritional recommendations and levels of evidence for those caring for infants with single ventricle physiology.     

Stent Dilatation of a Right Ventricle to Pulmonary Artery Conduit in a Postoperative Patient with Hypoplastic Left Heart Syndrome

A 10‐day‐old child with hypoplastic left heart syndrome (HLHS) underwent first‐stage palliation for HLHS, Norwood procedure with a Sano modification, i.e., placement of a right ventricular to pulmonary artery (RV‐PA) conduit. The patient developed progressively worsening systemic oxygen desaturation in the immediate postoperative period. Stenosis of the proximal RV‐PA conduit was diagnosed by echocardiography. In the catheterization laboratory stent placement in the conduit was performed. This resulted in increased systemic oxygen saturation. The patient was eventually discharged from the hospital with adequate oxygen saturations.     

胎儿超声心动图诊断左心发育不良综合征13例报道

目的:探讨应用胎儿超声心动图对左心发育不良综合征(HLHS),诊断方法及诊断价值。方法:对胎儿超声心动图检出的13例HLHS胎儿的超声资料与病理解剖结果对照,分析胎儿超声心动图诊断要点。结果:经病理证实为HLHS 13例,均与超声诊断结果一致,超声诊断正确率100%。13例HLHS患儿中,Ⅰ型2例(15.4%),Ⅱ型5例(38.4%),Ⅲ型2例(15.4%),Ⅳ型4例(30.8%)。超声提示13例均显示左、右心系统比例极端不对称,左心室小、发育不良,右心系统比例增大,主肺动脉内径比例失调,主肺动脉血流方向呈反向。结论:产前胎儿超声心动图作为一种安全、便捷、经济及有效的检查方法,对胎儿左心发育不良综合征的检出具有重要的价值。     

Echocardiographic Parameters that Predict Outcome in Aortic Atresia Patients Undergoing Comprehensive Stage II Procedure

Background. The hybrid procedure is one option for palliating patients with hypoplastic left heart syndrome. As experience increases with this palliation, the physiology and its influence on outcome can be better assessed. The goal of this study was to determine if echocardiographic parameters correlate with post-operative variables in patients with aortic atresia undergoing the comprehensive Stage II procedure. Methods. Retrospective chart review on all patients with aortic atresia, who underwent the comprehensive Stage II procedure from January 2002 to December 2008, was performed. Echocardiographic indices were evaluated and correlations were made with peri-operative and hospital variables. Pair-wise Pearson's correlation tests were used to analyze the associations between continuous measures. Results. Thirty-four patients met inclusion criteria. Age at comprehensive Stage II procedure was 0.45 ± 0.13 years and body surface area was 0.31 ± 0.04 m². Right ventricle (RV) percentage change was 45 ± 10%, eccentricity index was 1.96 ± 0.45, estimated systemic cardiac output was 7.68 ± 2.56 L/min/m² and estimated effective systemic cardiac output was 5.15 ± 2.24 L/min/m². Retrograde patent ductus arteriosu (PDA) velocity time integral (VTI) correlated with log pre-bypass lactate and maximum lactate (r = 0.53, 0.44). PDA regurgitant fraction correlated with log post-bypass lactate, length of intubation, and urine output on day four (r = 0.39, 0.46, −0.37). RV percentage change correlated with log pre-bypass lactate, and urine output on days four and five (r =−0.38, 0.43, 0.54). No echocardiographic parameter predicted renal or liver insufficiency, dialysis, extracorporeal membrane oxygenation use, or hospital death. Conclusion. Retrograde PDA VTI and RV percent change correlated with some peri-operative variables though no echocardiographic parameter was associated with any major morbidities or mortality. Newer echocardiographic techniques may better predict comprehensive stage II outcomes.     

Staged Single‐ventricle Palliation in 2011: Outcomes and Expectations

Outcomes for staged palliation for single‐ventricle heart disease have improved over the past two decades. As outcomes improve, parental expectations for survival and quality of life have risen accordingly. Nevertheless, the number of interventions and complications these patients must endure remain high. The final surgical destination of the single‐ventricle patient, the total cavopulmonary connection (or Fontan operation) successfully separates systemic venous and pulmonary venous blood flow but does so at great cost. Fontan patients remain at significant risk of complications despite what are perceived to be “favorable” hemodynamics. The outcomes in this population are discussed in this review, with particular attention to the history behind our current strategies as well as to recent salient studies.     

Acute Interventions for Stenosed Right Ventricle‐Pulmonary Artery Conduit Following the Right‐Sided Modification of Norwood‐Sano Procedure

Introduction. The Norwood stage 1 procedure was modified by Sano with right ventricle‐pulmonary artery (RV–PA) conduit replacing BT shunt. In our institution, this has been further modified by placing the conduit from the RV outflow tract to the right side of the neo‐aorta. Patients and Methods. Between April 2002 and October 2008, 227 modified Norwood procedures were performed. Eighteen had the Sano modification with the conduit to the left of the neo‐aorta whereas 209 had the right‐sided modification, which is the study population. A total of 18 (8.6%) patients presented with cyanosis due to conduit stenosis with median age 4 months and median weight 6.3 kg. Results. Twelve patients underwent transcatheter stent placement in stenosed RV–PA conduit. A total of 16 coronary stents were implanted in 12 patients with 4 patients each receiving 2 stents. The mean saturations increased from 60% to 74%. There was one late mortality which was non‐procedure related. Five patients treated with surgical take down of the RV–PA conduit and creation of a cavo‐pulmonary shunt, whilst one patient had replacement of RV–PA conduit. There were no early postoperative deaths. The mean saturations improved from 54% to 75%. Conclusions. The RV–PA conduit stenosis is a life‐threatening complication after the modified Norwood Stage I procedure. This may require urgent surgery to replace the conduit or to perform a cavo‐pulmonary shunt but as an alternative, transcatheter stent placement can be used with equal effectiveness and with a low risk of complications. The catheter approach is less invasive and the results show that it is an excellent option to relieve the stenosis even in the right‐sided RV–PA conduit.