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.     

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.     

A comparison of treatment strategies for hypoplastic left heart syndrome using decision analysis

OBJECTIVES: We sought to identify the optimal treatment strategy for hypoplastic left heart syndrome (HLHS). BACKGROUND: Surgical treatment of HLHS involves either transplantation (Tx) or staged palliation of the native heart. Identifying the best treatment for HLHS requires integrating individual patient risk factors and center-specific data. METHODS: Decision analysis is a modeling technique used to compare six strategies: staged surgery; Tx; stage 1 surgery as an interim to Tx; and listing for transplant for one, two, or three months before performing staged surgery if a donor is unavailable. Probabilities were derived from current literature and a dataset of 231 patients with HLHS born between 1989 and 1994. The goal was to maximize first-year survival. RESULTS: If a donor is available within one month, Tx is the optimal choice, given baseline probabilities; if no donor is found by the end of one month, stage 1 surgery should be performed. When survival and organ donation probabilities were varied, staged surgery was the optimal choice for centers with organ donation rates < 10% in three months and with stage 1 mortality <20%. Waiting one month on the transplant list optimized survival when the three-month organ donation rate was > or =30%. Performing stage 1 surgery before listing, or performing stage 1 surgery after an unsuccessful two- or three-month wait for transplant, were almost never optimal choices. CONCLUSIONS: The best strategy for centers that treat patients with HLHS should be guided by local organ availability, stage 1 surgical mortality and patient risk factors.     

Electrolytically detachable coils for closure of a modified baffle fenestration in patients with hypoplastic left heart syndrome (HLHS)

Today total cavopulmonary connection (TCPC) is the treatment of choice for long term palliation of patients with hypoplastic left heart syndrome (HLHS). We report a new, relatively simple and effective method to occlude a modified baffle (TCPC) fenestration via the left hepatic vein in two patients with HLHS with the use of electrolytically detachable platinum microcoils after a failed tourniquet closure.     

Evolving strategies and improving outcomes of Norwood stage one reconstruction: a decade experience of a single institution in Taiwan

AIM: The aim of this study was to define the improvement in short-term outcome and risk factors of Norwood stage one reconstruction for hypoplastic left heart syndrome (HLHS) in Taiwan, after implementing new perioperative management strategies. METHODS: Data were retrieved from a retrospective chart review of patients with HLHS treated between July 1997 and July 2007. Since we implemented new perioperative strategies in 2004, we divided our patients into two groups, early era (1997 - 2003) and late era (2004 - 2007), and compared the outcome. RESULTS: We enrolled 48 patients. In the early era group (n = 28), the diagnosis was confirmed by cardiac catheterization and controlled ventilation was used to manipulate the balance between systemic and pulmonary blood flow. The survival rate was only 17.9 % (5/28). Surgery was performed at 15.2 +/- 10.7 days, which was significantly later than in the late era group (4.6 +/- 4.0 days, n = 20). A lower preoperative shock and more prenatal diagnoses were recorded for the late era group. RV-PA conduit was used in 17 patients in the late era group of which 12 (70.6 %) survived to be discharged from hospital. The risk factor was significant TR (triscupid regurgitation). CONCLUSIONS: With our contemporary perioperative management and change in surgical strategy, survival after first-stage palliation has improved. We believe that our HLHS experience is valuable for low volume centers and also for Asian cohorts.     

Coil‐occlusion of the left ventricle as emergency treatment in failing stage I palliation for hypoplastic left heart syndrome with sinusoids

We report on a patient with hypoplastic left heart syndrome (HLHS), ventricular septal defects, and coronary sinusoids who suffered recurrent myocardial ischemic events that required cardiopulmonary resuscitation after stage I palliation. We identified the main reason to be a steal phenomenon of blood from the left coronary artery via the sinusoids into the rudimentary left ventricle and across the septal defects into the right ventricle. To limit this coronary steal phenomenon, we successfully performed transcatheter closure of the left ventricle with implantation of three Microplex Terumo®‐Coils. This led to the patient's clinical stabilization. Stage II surgery took place at the age of 5 months without difficulty. In the setting of a symptomatic neonate with HLHS and ventriculocoronary connections, embolization of the left ventricle is a feasible interventional therapy. © 2012 Wiley Periodicals, Inc.     

Chirurgische Behandlung des hypoplastischen Linksherzsyndroms

Hypoplastic left heart syndrome (HLHS) represents an anatomical spectrum of congenital disease with varying degrees of underdevelopment of the left-sided cardiac structures (Figure 1). The outlook for children born with HLHS, an otherwise highly lethal malformation, has improved with increasing experience with reconstructive techniques. This report represents a detailed analysis of the overall risk and mid-term results for a group of 39 consecutive neonates with HLHS referred to our hospital over a 5-year period between January 1994 and November 1998. Twenty-six patients were treated with a Norwood reconstructive procedure (Figure 2). One patient received a cardiac transplant at the request of the parents. Another patient with aortic atresia, ventricular septal defect and normal left ventricle underwent biventricular repair. Eleven patients were not eligible for surgical treatment due to a number of reasons (Table 1). In 26 neonates (9 girls, 17 boys; mean age 9.1 [4 to 42] days) staged reconstruction by Norwood's procedure was performed. The hospital mortality in the first stage of the Norwood procedure was 23% (6/26). Sixteen of the 20 long-term survivors underwent the bidirectional cavopulmonary anastomosis (hemi-Fontan; Figure 3) at a median age of 7.6 (3 to 14) months. All children survived. Up to now, 3 infants received the complete Fontan operation (Figure 4) at 2 years of age. No late death occurred. In 18 out of the 20 survivors neuro-developmental outcome and exercise performance were within the range of normals. Staged surgical palliation represents a realistic therapeutic option for neonates born with HLHS. At this intermediate stage of follow-up exercise performance and quality of life are satisfactory.     

Atrial septal interventions in patients with hypoplastic left heart syndrome

Objectives: To report an institutional experience performing percutaneous atrial septal interventions in patients with hypoplastic left heart syndrome (HLHS). Background: The success of the Hybrid approach in palliating patients with HLHS is crucially dependant on relieving any significant interatrial restriction. Data on transcatheter interventions to relieve atrial septal restrictions in patients with HLHS are limited. Methods: We retrospectively reviewed 67 transcatheter atrial septal interventions that were performed between July 2002 and September 2007 in 56 patients with HLHS. The median weight was 3.35 kg. About 10.7% of patients had an intact atrial septum. Balloon atrial septostomy (BAS) was used in 77.6% of procedures, additional techniques in 35.8% of procedures. Patients were divided into those with standard atrial septal anatomy (group A, n = 33) and those with complex atrial septal anatomy (group B, n = 23). Results: The mean trans‐septal gradient was reduced significantly from 7 mm Hg to 1 mm Hg with the median time to discharge being 3.5 days. Major adverse events were seen in 8.9% of procedures, whereas minor adverse events occurred in 26.8% of procedures. Adverse events were significantly less common in patients with standard atrial septal anatomy, compared to those with complex atrial septal anatomy (25.6% versus 50.0%). About 19.6% patients required repeated atrial septal interventions. Survival up to and including Comprehensive stage II palliation was 73% group A, and 57% in group B. Conclusions: With utilization of appropriate techniques and equipment, atrial septal interventions in HLHS can be performed successfully in virtually all patients. Complex atrial septal anatomy is technically challenging and has a higher incidence of procedural adverse events. In the majority of patients, standard BAS can be performed safely, and is usually the only intervention required to achieve adequate relief of atrial septal restriction until Comprehensive stage II palliation. © 2008 Wiley‐Liss, Inc.     

Hypoplastic Left Heart Syndrome Across the Lifespan: Clinical Considerations for Care of the Fetus,Child, and Adult

Hypoplastic left heart syndrome (HLHS) is the most common anatomic lesion in children born with single-ventricle physiology and is characterised by the presence of a dominant right ventricle and a hypoplastic left ventricle along with small left-sided heart structures. Diagnostic subgroups of HLHS reflect the extent of inflow and outflow obstruction at the aortic and mitral valves, specifically stenosis or atresia. If left unpalliated, HLHS is a uniformly fatal lesion in infancy. Following introduction of the Norwood operation, early survival has steadily improved over the past 4 decades, mirroring advances in operative and perioperative management as well as reflecting refinements in patient surveillance and interstage clinical care. Notably, survival after staged palliation has increased from 0% to a 5-year survival of 60%-65% for children in some centres. Despite the prevalence of HLHS in childhood with relatively favourable surgical outcomes in contemporary series, this cohort is only now reaching early adult life and longer-term outcomes have yet to be elucidated. In this article we focus on contemporary clinical management strategies for patients with HLHS across the lifespan, from fetal to adult life. Nomenclature and diagnostic considerations are discussed and current literature pertaining to putative genetic etiologies is reviewed. The spectrum of fetal and paediatric interventional strategies, both percutaneous and surgical, is described. Clinical, patient-reported, and neurodevelopmental outcomes of HLHS are delineated. Finally, note is made of current areas of clinical uncertainty, and suggested directions for future research are highlighted.     

Two-dimensional echocardiographic assessment of right ventricular function as a predictor of outcome in hypoplastic left heart syndrome

This study was undertaken to assess the importance of right ventricular function at the time of initial presentation on early and intermediate outcome in patients with hypoplastic left heart syndrome (HLHS). Several studies have attempted to define physiologic risk factors for poor early outcome following the Norwood palliation for HLHS. No clinical or hemodynamic factors including right ventricular function have been found to reliably predict Norwood I operative survival. The relation between initial ventricular function and later survival has not been investigated. To assess the importance of right ventricular (RV) function at the time of initial presentation on outcome in patients with HLHS, systolic function was determined by qualitative and quantitative methods in 60 consecutive patients before surgical intervention. The effects on stage I operative survival, survival to stage II, and overall survival were analyzed. Initial RV function did not impact on stage I survival. However, analysis of later outcome of the stage I survivors showed that those with prestage I RV dysfunction had significantly greater mortality before stage II. Actuarial survival 18 months after Norwood surgery was 93% for patients with initially normal RV function compared with 47% for those with abnormal function (p = <0.005). The relative risk for later mortality was approximately 11 times greater for patients with initial RV dysfunction. Thus, RV dysfunction identifiable soon after initial presentation does not impact on early survival after Norwood I operation for HLHS. Intermediate and overall survival, however, is significantly decreased in patients with initially diminished RV function.     

Outcome and resource utilization of infants born with hypoplastic left heart syndrome in the intermountain west

The objective of the present study was to characterize the outcomes and resource utilization of all infants born with hypoplastic left heart syndrome (HLHS) in the Intermountain West. This was a retrospective cohort study of all infants born with HLHS in the Intermountain West from January 1995 and January 2010. The cohort was divided into 3 eras: era 1, 1995 to 1999; era 2, 2000 to 2004; and era 3, 2005 to 2010. Cox proportional hazards regression analysis was performed to assess mortality. The lifetime hospitalization days and charges were also determined. Of the 245 infants identified, 65% were male infants and 172 (70%) underwent Stage 1 palliation. The transplant-free survival rate for the entire cohort was 33% at 14 years. The 1-year transplant-free survival rate for the surgical cohort was 60% in era 3. The infants whose initial presentation included shock, restrictive or intact atrial septum, chromosomal defects, or multiorgan dysfunction had an increased risk of death. A recent era of birth, greater birthweight, and older gestational age were associated with improved survival. The factors associated with mortality after stage 1 included surgical procedure type (Blalock-Taussig vs Sano shunt, hazard ratio 2.1), requirement for postoperative extracorporeal membrane oxygenation (hazard ratio 4.2), postoperative renal dysfunction (hazard ratio 3.0), anomalous pulmonary venous return (hazard ratio 2.9), and moderate or greater tricuspid valve regurgitation at any point (hazard ratio 2.0). For patients who had undergone stage 1, 2, or 3 palliation, the median cumulative lifetime hospitalization was 32, 48, and 65 days, and the median cumulative lifetime charges for hospitalization were $201,812, $253,183, and $296,213, respectively. In conclusion, although hospital-based studies of HLHS have shown significantly improved survival after surgical palliation, population-based studies have shown that HLHS continues to have a high mortality and high resource utilization.     

Postoperative hemodynamics after Norwood palliation for hypoplastic left heart syndrome

Hemodynamics after Norwood palliation for hypoplastic left heart syndrome (HLHS) have been incompletely characterized, although emphasis has been placed on the role that an excess pulmonary-to-systemic blood flow ratio (Qp/Qs) may play in causing hemodynamic instability. Studies suggest that maximal oxygen delivery occurs at a Qp/Qs < 1. However, it remains unclear to what extent cardiac output can increase with increasing pulmonary perfusion. One approach is to use the oxygen excess factor omega, an index of systemic oxygen delivery, and compare omega with measured Qp/Qs. We measured Qp/Qs and omega in neonates after Norwood palliation for HLHS, and determined how they were related. In addition, we determined the temporal course of surrogate indexes of systemic perfusion in the early postoperative period. Arteriovenous oxygen saturation difference, blood lactate, and omega were recorded on admission and every 3 to 12 hours for 2 days in 18 consecutive infants with HLHS or variant after Norwood palliation. Three infants required extracorporeal membrane oxygenation (ECMO) 6 to 9 hours after admission. These infants had higher Qp/Qs, blood lactate, arteriovenous oxygen saturation difference, and lower omega than non-ECMO patients. In non-ECMO patients between admission and 6 hours, omega decreased significantly despite no appreciable change in Qp/Qs. We conclude that: (1) Oxygen delivery is significantly decreased at 6 postoperative hours unrelated to Qp/Qs. This modest decline in oxygen delivery is insufficient to compromise tissue oxygenation. (2) Patients requiring ECMO have significant derangements in oxygen delivery.     

Percutaneous coronary intervention in a 3‐year‐old with hypoplastic left heart syndrome

Mortality outcomes associated with surgical palliation for hypoplastic left heart syndrome (HLHS) have continued to improve with time. Despite these advances, interstage mortality continues to be difficult to predict and prevent. This case report details a 3‐year‐old boy with HLHS palliated with a Glenn shunt who presented with acute myocardial infarction due to near total occlusion of the distal right coronary artery. He was treated by thrombus aspiration with good clinical response. This is the first report of thrombus aspiration in complex pediatric congenital heart disease and demonstrates the utility of collaboration between congenital and adult cardiologists. © 2015 Wiley Periodicals, Inc.     

Transcatheter device occlusion of the left ventricular outflow tract as treatment for severe aortic regurgitation in hypoplastic left heart syndrome

Aortic regurgitation is a rare but dangerous condition in patients with hypoplastic left heart syndrome (HLHS). We report the case of a 2‐year‐old girl with HLHS with aortic/mitral stenosis (AS‐MS) subtype after stage II palliation via the bilateral bidirectional Glenn (BBDG) procedure, when aortic regurgitation presented with the clinical manifestation of a paroxysmal coronary steal phenomenon provoked by exertion. Transcatheter closure of the left ventricular outflow tract (LVOT) with an Amplatzer^TM Vascular Plug 4 minimized and finally abolished aortic reflux without compromising either coronary perfusion or atrioventricular (AV) conduction. Midterm follow‐up over 3 years revealed complete disappearance of her symptoms, and follow‐up catheterization displayed complete LVOT closure. This case illustrates the promising potential of modern cardiac catherization techniques in certain cases and emphasizes that precise assessment of the native aorta and coronary system is critical in HLHS patients. © 2015 Wiley Periodicals, Inc.     

Hypoplastic left heart syndrome associated with congenital right-sided diaphragmatic hernia and omphalocele.

Congenital diaphragmatic hernia (CDH) is associated with a variety of cardiac anomalies. However, its association with hypoplastic left heart syndrome (HLHS) is rare. We treated a female newborn with CDH, HLHS, and omphalocele. The operation for omphalocele and the diaphragmatic defect was successful, although the patient died of cardiac failure after Norwood's operation for HLHS. To our knowledge, this is the first reported case with a combination of these three major anomalies: CDH, HLHS, and omphalocele.     

Hand‐held echocardiography in children with hypoplastic left heart syndrome

Background: When performed by cardiologists, hand‐held echocardiography (HHE) can assess ventricular systolic function and valve disease in adults, but its accuracy and utility in congenital heart disease is unknown. In hypoplastic left heart syndrome (HLHS), the echocardiographic detection of depressed right ventricular (RV) systolic function and higher grade tricuspid regurgitation (TR) can identify patients who are at increased risk of morbidity and mortality and who may benefit from additional imaging or medical therapies.
Methods: Children with HLHS after Stage I or II surgical palliation (Norwood or Glenn procedures) were prospectively enrolled. Subjects underwent HHE by a pediatric cardiologist on the same day as standard echocardiography (SE). Using 4‐point scales, bedside HHE assessment of RV systolic function and TR were compared with blinded assessment of offline SE images. Concordance correlation coefficient (CCC) was used to evaluate agreement.
Results: Thirty‐two HHEs were performed on 15 subjects (Stage I: n = 17 and Stage II: n = 15). Median subject age was 3.4 months (14 days‐4.2 years). Median weight was 5.9 kg (2.6‐15.4 kg). Bedside HHE assessment of RV systolic function and TR severity had substantial agreement with SE (CCC = 0.80, CCC = 0.74, respectively; P < .001). HHE sensitivity and specificity for any grade of depressed RV systolic function were 100% and 92%, respectively, and were 94% and 88% for moderate or greater TR, respectively. Average HHE scan time was 238 seconds.
Conclusions: HHE offers a rapid, bedside tool for pediatric cardiologists to detect RV systolic dysfunction and hemodynamically significant TR in HLHS.     

Preoperative management of pulmonary venous hypertension in hypoplastic left heart syndrome with restrictive atrial septal defect

A severely restrictive atrial septal defect (ASD) in neonates with hypoplastic left heart syndrome (HLHS) results in pulmonary venous hypertension, pulmonary edema, and intractable hypoxia. Between January 1983 and June 1998, 21 of 355 neonates presenting with HLHS (5.9%) underwent cardiac catheterization at median age 1 day (range 0 to 25), for creation or enlargement of a restrictive or absent interatrial communication. One patient died during preliminary angiography. Three underwent blade septostomy with 2 procedure-related deaths, and 1 had balloon atrial septostomy (BAS); all 4 died before surgical intervention. Fifteen underwent Brockenbrough atrial septoplasty with transatrial needle puncture and serial balloon dilations of the new ASD, 5 after unsuccessful BAS. The most recent patient had a stent placed across the atrial septum after transatrial needle puncture. In the 16 patients treated with septoplasty or stent, oxygen saturation increased from 50 +/- 4% to 83 +/- 2% (p <0.0001) and transatrial pressure gradient decreased from 16 +/- 1 to 6 +/- 1 mm Hg (p <0.0001). One patient died awaiting transplantation, supportive care only was requested in 1, and 14 underwent stage 1 palliation. Eight of 14 (57%) survived to hospital discharge. Six of 7 (86%) survived bidirectional Glenn and the 3 who have undergone fenestrated Fontan are alive. In neonates with HLHS, a restrictive ASD resulting in profound cyanosis demands urgent intervention. BAS is frequently unsuccessful and blade septostomy has high mortality. Pulmonary venous hypertension can be adequately relieved by Brockenbrough atrial septoplasty or stenting, allowing stabilization before reconstructive surgery or while awaiting transplant.     

Growth characteristics of the aortic arch after the Norwood operation

Objectives. We sought to characterize the growth of the reconstructed aortic arch after the Norwood operation (NO).

Background. The first stage of surgical palliation of hypoplastic left heart syndrome (HLHS), the NO, includes augmentation of the aortic arch with homograft. Growth characteristics of the reconstructed aortic arch, which is comprised of both native aortic tissue and homograft, have not been characterized.

Methods. Retrospectively, we examined the serial echocardiograms of 50 patients with HLHS who underwent NO to determine the diameter of the reconstructed transverse arch. Measurements were taken immediately after NO and at two other points (1 to 11 years of age). In addition, the autopsy specimens of 10 other patients with HLHS who underwent NO were examined to determine the contribution of native aortic tissue to the overall size of the reconstructed arch at the time of death (12 to 34 months).

Results. The diameter of the transverse aorta increased after NO in all subjects. Its rate of growth paralleled that seen in the normal population, though the reconstructed arch had a significantly larger diameter throughout childhood. Examination of autopsy specimens demonstrated a mean increase in circumference of the native aortic tissue of 0.67 cm (p value <0.01), whereas there was no significant change in homograft circumference.

Conclusions. After reconstruction of the aortic arch in HLHS, the diameter of the arch continues to increase throughout childhood, and this increase is due to growth of the native aortic tissue.     

Hybrid transcatheter tricuspid valve‐in‐valve placement of an Edwards Sapien XT valve in palliated hypoplastic left heart syndrome

Tricuspid regurgitation in the context of hypoplastic left heart syndrome (HLHS) carries a significant burden of both mortality and morbidity. We report successful hybrid trans‐atrial implantation of an Edwards Sapien XT valve into a dysfunctional bioprosthetic valve in the tricuspid position of a 9‐year‐old patient with HLHS. There was an immediate fall in the Fontan pressures combined with significant clinical improvement and the valve continues to function well nearly 3 years post implant. To the best of our knowledge, this is the first described implantation of a transcatheter valve in this specific position and anatomy.