Comprehensive Approach to the Management of Patients with HLHS: Analysis of 100 Consecutive Neonates.

BACKGROUND: We report our comprehensive approach to patients with hypoplastic left heart syndrome (HLHS) and describe our outcomes in 100 consecutive neonates. METHODS: One-hundred consecutive neonates (2015-2023) were stratified into 3 pathways: Pathway(1): 77/100=77% were standard-risk and underwent initial Norwood (Stage 1). Pathway(2): 10/100=10% were high-risk with noncardiac risk factors and underwent initial Hybrid Stage 1. Pathway(3): 13/100=13% were high-risk with cardiac risk factors: 10 underwent initial Hybrid Stage 1 + ventricular assist device insertion (HYBRID+VAD), while 3 underwent primary transplantation. RESULTS: One-year mortality=9/100=9%. Pathway(1): Operative Mortality for initial Norwood (Stage 1)=2/77=2.6%. Of 75 survivors of Norwood (Stage 1): 72 underwent successful Glenn, 2 underwent successful biventricular repair, and 1 underwent successful cardiac transplantation. Pathway(2): Operative Mortality for initial Hybrid Stage 1 without VAD=1/10=10%. Of 9 survivors of Hybrid (Stage 1): 4 underwent successful cardiac transplantation, 2 died while awaiting cardiac transplantation, 3 underwent Comprehensive Stage 2 (with 1 death), and 1 underwent successful biventricular repair. Pathway(3): Of 10 HYBRID+VAD: 7/10=70% underwent successful cardiac transplantation and are alive today and 3/10=30% died on VAD while awaiting transplantation. Median VAD support time=134 days (range=56-226). (Two of three patients who were bridged-to-transplant with prostaglandin underwent successful transplantation and one died while awaiting transplantation.) CONCLUSIONS: A comprehensive approach to the management of patients with HLHS is associated with Operative Mortality after Norwood of 2/77=2.6% and an overall one-year mortality of 9/100=9%. 10/100 patients=10% were stabilized with HYBRID+VAD while awaiting transplantation. VAD facilitates survival on the waiting list during prolonged wait times.

Outcomes of Children Supported With Pulsatile Paracorporeal Ventricular Assist Device: Congenital Versus Acquired Heart Disease.

BACKGROUND: We reviewed the outcomes of 82 consecutive pediatric patients (less than 18 years of age) supported with the Berlin Heart ventricular assist device (VAD), comparing those with congenital heart disease (CHD; n = 44) with those with acquired heart disease (AHD; n = 37). METHODS: The primary outcome was mortality after VAD insertion. Kaplan-Meier methods and log-rank tests were used to assess group differences in long-term survival. RESULTS: Forty-four CHD patients were supported (age: median = 65 days, range = 4 days-13.3 years; weight [kg]: median = 4, range = 2.4-42.3). Ten biventricular CHD patients were supported with eight biventricular assist devices (BiVADs), one left ventricular assist device (LVAD) only, and one LVAD converted to BiVAD, while 34 univentricular CHD patients were supported with single ventricle-ventricular assist devices (sVADs). In CHD patients, duration of VAD support was [days]: median = 134, range = 4-554. Of 44 CHD patients, 28 underwent heart transplantation, 15 died on VAD, and one was still on VAD. Thirty-seven AHD patients were supported (age: median = 1.9 years, range = 27 days-17.7 years; weight [kg]: median = 11, range = 3.1-112), including 34 BiVAD and 3 LVAD. In AHD patients, duration of VAD support was [days]: median = 97, range = 4-315. Of 37 AHD patients, 28 underwent transplantation, three died on VAD, five weaned off VAD (one of whom underwent heart transplantation 334 days after weaning), and one was still on VAD. One-year survival after VAD insertion was 59.9% (95% CI = 46.7%-76.7%) in CHD and 88.6% (95% CI = 78.8%-99.8%) in AHD, P = .0004. Five-year survival after VAD insertion was 55.4% (95% CI = 40.8%-75.2%) in CHD and 85.3% (95% CI = 74.0%-98.2%) in AHD, P = .002. CONCLUSIONS: Pulsatile VAD facilitates bridge-to-transplantation in neonates, infants, and children with CHD; however, survival after VAD insertion is worse in patients with CHD than in patients with AHD.

Elevated Aortic Stiffness after Pediatric Heart Transplantation.

In adults, arterial stiffness has been linked to the development of target end-organ damage, thought to be related to abnormal transmission of pulse pressure. Increased arterial stiffness and endothelial dysfunction have been hypothesized to contribute to the development of microvascular dysfunction and coronary allograft vasculopathy (CAV), an important comorbidity after heart transplantation. However, little data exists regarding arterial stiffness in pediatric heart transplantation and its influence on development of coronary allograft vasculopathy is not well understood. We sought to assess aortic stiffness and distensibility in pediatric post-heart transplant patients. A prospective, observational study analyzing the ascending (donor tissue) and descending aorta (recipient tissue) using transthoracic echocardiographic M-mode measurements in patients aged < 21 years was conducted. Descending and ascending aorta M-modes were obtained from the subcostal long axis view, and the parasternal long axis view 3-5mm above the sinotubular junction, respectively. Two independent reviewers averaged measurements over 2-3 cardiac cycles, and Aortic Distensibility (AD) and Aortic Stiffness Index (ASI) were calculated using previously validated methods. We recruited 39 heart transplant (HT) patients and 47 healthy controls. Median end diastolic dimension of the ascending aorta (donor tissue) was significantly larger in the transplant group than the control group (1.92 cm vs. 1.74 cm, p = 0.01). Ascending aortic distensibility in post-transplant patients was significantly lower than in the control group (4.87 vs. 10.53, p < 0.001). Ascending aortic stiffness index was higher in the transplant patients compared to the controls (4.63 vs. 2.21, p < 0.001). There is evidence of altered ascending aortic distensibility and stiffness parameters in post-heart transplant patients. Further studies are required to assess its influence on complications like development of coronary artery vasculopathy.

Support with Single Ventricle-Ventricular Assist Device (sVAD) in Patients with Functionally Univentricular Circulation Prior to Fontan Operation.

Some patients with functionally univentricular circulation develop cardiac failure refractory to maximal management and are supported with a ventricular assist device (VAD). The purpose of this manuscript is to summarize our previous publications related to single ventricle-ventricular assist device (sVAD) support in patients with functionally univentricular circulation and to describe our current institutional approach at University of Florida to sVAD support in neonates, infants, and children prior to Fontan. Our programmatic philosophy at University of Florida is to strive to identify the minority of neonates with functionally univentricular circulation who are extremely high-risk prior to initiating staged palliation and to stabilize these neonates with primary preemptive sVAD in preparation for cardiac transplantation; our rationale for this approach is related to the challenges associated with failed staged palliation and subsequent bail-out sVAD support and transplantation. A subset of extremely high-risk neonates and infants with functionally univentricular ductal-dependent circulation undergo primary preemptive sVAD insertion and subsequent cardiac transplantation. Support with VAD clearly facilitates survival on the waiting list during prolonged wait times and optimizes outcomes after Norwood (Stage 1) by providing an alternative pathway for extremely high-risk patients. Therefore, the selective utilization of sVAD in extremely high-risk neonates facilitates improved outcomes for all patients with functionally univentricular ductal-dependent circulation. At University of Florida, our programmatic approach to utilizing sVAD support as a bridge to transplantation in the minority of neonates with functionally univentricular circulation who are extremely high-risk for staged palliation is associated with Operative Mortality after Norwood (Stage 1) Operation of 2.9% (2/68) and a one-year survival of 91.1% (82/90) for all neonates presenting with hypoplastic left heart syndrome (HLHS) or HLHS-related malformation with functionally univentricular ductal-dependent systemic circulation. Meanwhile, at University of Florida, for all 82 consecutive neonates, infants, and children supported with pulsatile paracorporeal VAD: Kaplan-Meier survival estimated one year after VAD insertion = 73.3% (95% confidence interval [CI] = 64.1-83.8%), and Kaplan-Meier survival estimated five years after VAD insertion = 68.3% (95% CI = 58.4-79.8%). For all 48 consecutive neonates, infants, and children at University of Florida with biventricular circulation supported with pulsatile paracorporeal VAD: Kaplan-Meier survival estimated one year after VAD insertion = 82.7% (95% CI = 72.4-94.4%), and Kaplan-Meier survival estimated five years after VAD insertion = 79.7% (95% CI = 68.6-92.6%). For all 34 consecutive neonates, infants, and children at University of Florida with functionally univentricular circulation supported with pulsatile paracorporeal sVAD: Kaplan-Meier survival estimated one year after VAD insertion = 59.7% (95% CI = 44.9-79.5%), and Kaplan-Meier survival estimated five years after VAD insertion = 50.5% (95% CI = 35.0-73.0%). These Kaplan-Meier survival estimates for patients supported with pulsatile paracorporeal VAD are better in patients with biventricular circulation in comparison to patients with functionally univentricular circulation both one year after VAD insertion (P=0.026) and five years after VAD insertion (P=0.010). Although outcomes after VAD support in functionally univentricular patients are worse than in patients with biventricular circulation, sVAD provides a reasonable chance for survival. Ongoing research is necessary to improve the outcomes of these challenging patients, with the goal of developing strategies where outcomes after sVAD support in functionally univentricular patients are equivalent to the outcomes achieved after VAD support in patients with biventricular circulation.

Ventricular assist device support in neonates and infants with a failing functionally univentricular circulation.

Some neonates with functionally univentricular hearts are at extremely high risk for conventional surgical palliation. Primary cardiac transplantation offers the best option for survival of these challenging neonates; however, waitlist mortality must be minimized. We have developed a comprehensive strategy for the management of neonates with functionally univentricular hearts that includes the selective use of conventional neonatal palliation in standard-risk neonates, hybrid approaches in neonates with elevated risk secondary to a noncardiac etiology, and neonatal palliation combined with insertion of a single ventricular assist device (VAD) in neonates with elevated risk secondary to a cardiac etiology. Here we describe our selection criteria, technical details, management strategies, pitfalls, and current outcomes for neonates with functionally univentricular hearts supported with a VAD. Our experience shows that extremely high-risk neonates with functionally univentricular hearts who are poor candidates for conventional palliation can be successfully stabilized with concomitant palliation and pulsatile VAD insertion while awaiting cardiac transplantation.

A Comprehensive Approach to the Management of Patients With HLHS and Related Malformations: An Analysis of 83 Patients (2015-2021).

Background: Some patients with hypoplastic left heart syndrome (HLHS) and HLHS-related malformations with ductal-dependent systemic circulation are extremely high-risk for Norwood palliation. We report our comprehensive approach to the management of these patients designed to maximize survival and optimize the utilization of donor hearts. Methods: We reviewed our entire current single center experience with 83 neonates and infants with HLHS and HLHS-related malformations (2015-2021). Standard-risk patients (n = 62) underwent initial Norwood (Stage 1) palliation. High-risk patients with risk factors other than major cardiac risk factors (n = 9) underwent initial Hybrid Stage 1 palliation, consisting of application of bilateral pulmonary bands, stent placement in the patent arterial duct, and atrial septectomy if needed. High-risk patients with major cardiac risk factors (n = 9) were bridged to transplantation with initial combined Hybrid Stage 1 palliation and pulsatile ventricular assist device (VAD) insertion (HYBRID + VAD). Three patients were bridged to transplantation with prostaglandin. Results: Overall survival at 1 year = 90.4% (75/83). Operative Mortality for standard-risk patients undergoing initial Norwood (Stage 1) Operation was 2/62 (3.2%). Of 60 survivors: 57 underwent Glenn, 2 underwent biventricular repair, and 1 underwent cardiac transplantation. Operative Mortality for high-risk patients with risk factors other than major cardiac risk factors undergoing initial Hybrid Stage 1 palliation without VAD was 0/9: 4 underwent transplantation, 1 awaits transplantation, 3 underwent Comprehensive Stage 2 (with 1 death), and 1 underwent biventricular repair. Of 9 HYBRID + VAD patients, 6 (67%) underwent successful cardiac transplantation and are alive today and 3 (33%) died while awaiting transplantation on VAD. Median length of VAD support was 134 days (mean = 134, range = 56-226). Conclusion: A comprehensive approach to the management of patients with HLHS or HLHS-related malformations is associated with Operative Mortality after Norwood of 2/62 = 3.2% and a one-year survival of 75/83 = 90.4%. A subset of 9/83 patients (11%) were stabilized with HYBRID + VAD while awaiting transplantation. VAD facilitates survival on the waiting list during prolonged wait times.

Palliation Plus Ventricular Assist Device Insertion in 15 Neonates and Infants With Functionally Univentricular Circulation.

BACKGROUND: We report 15 high-risk neonates and infants with functionally univentricular circulation stabilized with initial surgical palliation plus ventricular assist device (VAD) insertion (PALLIATION+VAD) in preparation for transplantation. METHODS: Fifteen functionally univentricular patients with ductal-dependent systemic circulation (8 hypoplastic left heart syndrome, 1 hypoplastic left heart syndrome-related malformation: 7 neonates, 2 infants) or ductal-dependent pulmonary circulation (6 hypoplastic right heart syndrome: 5 neonates, 1 infant) presented with anatomical and/or physiological features associated with increased risk for conventional univentricular palliation (large coronary sinusoids with ventricular-dependent coronary circulation, severe systemic atrioventricular valvar regurgitation, cardiogenic shock, or restrictive atrial septum). PALLIATION+VAD for patients with ductal-dependent systemic circulation was: VAD insertion plus application of bilateral pulmonary bands, stent placement in the arterial duct, and atrial septectomy, if needed. PALLIATION+VAD for patients with ductal-dependent pulmonary circulation was: VAD insertion plus stent placement in the arterial duct or systemic-to-pulmonary artery shunt with pulmonary arterioplasty, if needed. RESULTS: At PALLIATION+VAD, median age was 20 days (range, 4-143 days) and median weight was 3.47 kg (range, 2.43-4.86 kg). Ten patients (67%) survived and 5 patients (33%) died. All ten survivors are at home doing well after successful transplantation. Only 2 of 10 survivors (20%) required intubation >10 days after PALLIATION+VAD. Median length of VAD support for all 15 patients was 138 days (range, 56-226 days). CONCLUSIONS: High-risk neonates with functionally univentricular hearts who are suboptimal candidates for conventional palliation can be successfully stabilized with pulsatile VAD insertion along with initial palliation while awaiting cardiac transplantation; these patients may be extubated, enterally nourished, and optimized for transplantation while on VAD.

Combined Hybrid Procedure and VAD Insertion in 9 High-Risk Neonates and Infants With HLHS.

BACKGROUND: This report describes 9 high-risk neonates and infants with hypoplastic left heart syndrome (HLHS) who were stabilized with a combined hybrid approach and ventricular assist device (VAD) insertion (HYBRID+VAD) in preparation for heart transplantation. METHODS: A total of 9 patients with HLHS (7 neonates, 2 infants) presented with anatomic or physiologic features associated with an increased risk for conventional univentricular palliation with the Norwood operation (large coronary sinusoids or fistulas, severe tricuspid regurgitation, cardiogenic shock, restrictive atrial septum). These patients underwent combined VAD insertion (Berlin EXCOR, Berlin Heart, Inc, Berlin, Germany) and Stage 1 hybrid palliation (application of bilateral pulmonary bands, stent placement in the patent arterial duct, and atrial septectomy if needed). During this same era, at the Congenital Heart Center, University of Florida, Gainesville, Florida, 46 neonates underwent a Norwood operation, 4 neonates underwent a hybrid approach "Stage 1" without VAD, and 3 patients with HLHS were supported with prostaglandin while awaiting heart transplantation. RESULTS: At HYBRID+VAD insertion, the median age was 20 days (range, 13 to 143 days), and median weight was 3.25 kg (range, 2.43 to 4.2 kg). Six patients survive (67%), and 3 patients died (33%). Five survivors are at home doing well after successful heart transplantation, and 1 survivor is doing well in the intensive care unit on VAD support while awaiting transplantation. Only 1 of 6 survivors (16.7%) required intubation more than 10 days after HYBRID+VAD insertion. In 8 patients no longer undergoing VAD support, the median length of VAD support was 119.5 days (range, 56 to 196 days). CONCLUSIONS: High-risk patients with HLHS who are suboptimal candidates for Norwood palliation can be successfully stabilized with pulsatile VAD insertion along with hybrid palliation while awaiting cardiac transplantation. These patients may be extubated and optimized for transplantation while undergoing VAD support.

Risk factors associated with post-transplant superior caval vein stenosis in paediatric heart transplantation.

Superior caval vein stenosis is a known complication following paediatric heart transplantation. Herein, we sought to assess the incidence of superior caval vein stenosis and need for intervention in a single centre paediatric heart transplantation programme. A retrospective review was performed to identify variables associated with superior caval vein stenosis and need for intervention. Patients were identified based on angiographic and echocardiographic signs of superior caval vein stenosis. Of 204 paediatric heart transplantation recipients, 49 (24.0%) had evidence of superior caval vein stenosis with no need for catheter intervention and 12 (5.9%) had superior caval vein stenosis requiring catheter intervention. Overall, patients with superior caval vein stenosis with and without intervention had more cavopulmonary anastomosis (41.7%; 20.4%), pre-transplant superior caval vein procedures (41.7%; 28.6%), and bicaval approach (100.0%; 98.0%), compared to the group with no stenosis (11.9% and p = 0.015, 12.6% and p = 0.004, 73.4% and p < 0.001, respectively). Smaller recipients and donors were more likely to need intervention. Intervention was also seen more frequently in recipients who were younger at diagnosis (4.7 years) compared to non-intervention (13.3 years; p = 0.040). Re-intervention was required in 16.7% patients (n = 2) and was not associated with any complications.

Thenar Muscle Oxygen Saturation Levels: A Surrogate for Central Venous Oxygen Saturation?

PURPOSE: Shock is associated with increased tissue oxygen extraction. Near-infrared spectroscopy-derived thenar muscle tissue oxygenation (StO2) levels can provide an estimate of the oxygen supply-demand balance at the tissue level. We hypothesized that thenar StO2 levels would correlate with central venous oxygen saturation (ScvO2) levels, the gold standard for global tissue oxygen extraction in the body. METHODS: We prospectively enrolled 60 pediatric subjects admitted to pediatric intensive care unit or who underwent cardiac catheterization from September 2015 to March 2018. Thenar StO2 levels were measured using the InSpectra StO2 probe. Concurrent measurements of ScvO2 and peripheral tissue oxygenation (StO2) were achieved through simultaneous testing. For ScvO2, a central line placed in the superior vena cava was utilized for serum specimen collection, while the InSpectra probe recorded StO2 measurements from the thenar eminence of the patient's right hand. RESULTS: Sixty observations of thenar StO2 and ScvO2 levels were derived from 60 subjects. Mean thenar StO2 levels were 74.72 ± 11.18% and displayed significant correlation with paired ScvO2 measurements ( m = 72.17 ± 9.77%; ρ = 0.317, P = .018). Correlation was much more significant in subjects who were not on mechanical ventilatory support as opposed to those who were on it ( ρSORA = 0.496, PSORA = .003, vs ρVENT = 0.161, PVENT = .433). A thenar StO2 of 73% had a sensitivity of 80% and a specificity of 77.8% in predicting an ScvO2 of less than 65%. CONCLUSION: This is the first study to report correlation of thenar StO2 and ScvO2 levels in children. Our study results show a significant correlation between these levels. Thenar StO2 measurements may have a role in the bedside management of critically ill children in whom ScvO2 monitoring is not available.

Effect of prenatal diagnosis on hospital costs in complete transposition of the great arteries.

OBJECTIVE: Prenatal diagnosis of congenital heart disease (CHD) is associated with improved clinical outcomes, yet its impact on the cost of hospitalization is not well described. We hypothesized that prenatal diagnosis of complete transposition of the great arteries (d-TGA) results in lower total hospital costs compared with postnatal diagnosis. METHODS: Retrospective analysis of infants with d-TGA repaired at our center from July 2006 to 2014. Total charges from initial hospitalization until discharge were converted to costs using the cost-to-charge ratio and then converted into 2016 dollars using the consumer price index. A direct cost comparison from the hospital perspective was performed between groups. A secondary analysis included the cost of prenatal diagnosis. RESULTS: Thirty-three infants with d-TGA were identified; 8 with and 25 without prenatal diagnosis. There was no difference in baseline characteristics. Mean direct cost of hospitalization was higher in infants without prenatal diagnosis ($108 014 ± $51 305 vs $88 305 ± $22 896, P = .31). On secondary analysis, the cost of prenatal diagnosis was negligible compared with total hospital cost. CONCLUSIONS: Total cost of initial hospitalization was higher for infants without prenatal diagnosis of d-TGA. Prenatal diagnosis not only improves clinical outcomes but may also be cost saving in the current era of increasing health care costs.

Hypoplastic left heart in Turner's syndrome: a primary indication for transplant?

Survival for hypoplastic left heart syndrome patients following the Norwood procedure is 71-90%. Mortality in patients with Turner's syndrome and hypoplastic left heart syndrome after conventional palliation (Norwood operation) has been reported as high as 80%. This questions the approach of traditional staged palliation. Here, we report a patient with hypoplastic left heart syndrome and Turner's syndrome bridged to orthotopic heart transplantation following a hybrid procedure.

Maternal hyperoxygenation: A potential therapy for congenital heart disease in the fetuses? A systematic review of the current literature.

OBJECTIVES: To assess efficacy, safety, outcomes, and intrauterine complications following maternal hyperoxygenation (MH) therapy in fetuses with congenital heart disease (CHD). METHODS: A systematic review was performed following an electronic search of databases. Articles were published before January 1, 2017, in an English-language and non-English-language journals (with English translations), and included human fetuses and expectant mothers with a fetal diagnosis of CHD who received MH. RESULTS: Ninety-six articles were identified; 72 were excluded and 24 full-text articles were reviewed. Only 9 articles met inclusion criteria and were analyzed. A total of 270 fetuses underwent MH therapy: 169 had CHD, and 101 had normal heart anatomies. Seven studies used fetal echocardiography, while 2 studies used cardiac magnetic resonance imaging (CMR). The mean gestational age at therapy was 33.4 weeks (26-38 weeks). Majority of MH protocols used 100% FiO2 with non-rebreather face mask at 8 L of flow, achieving 60%-70% FiO2 , or maternal PaO2 goal of 250 mm Hg. No significant adverse events were reported. Four studies reported increased size of the hypoplastic cardiac structures after MH. Three studies utilized acute MH to risk stratify hypoplastic left heart syndrome fetuses. Two studies assessed acute MH in the setting of CMR. CONCLUSION: The current evidence for MH therapy suggests an increase in pulmonary blood flow, and venous return, ductal flow, and heart dimensions in fetuses. MH has potential as a diagnostic and therapeutic tool in fetuses with CHD. Further randomized controlled trials are needed to ascertain whether MH therapy provides improved outcomes on fetuses with certain types of CHD.

Echocardiographic evaluation of ventricular assist devices in pediatric patients.

BACKGROUND: The use of ventricular assist devices (VADs) in children is challenging because of small patient size, frequent structural heart disease, and the need for biventricular assist devices. This report describes the role of echocardiography in the management of children supported by VADs. METHODS: A retrospective review of the records of all pediatric patients who underwent VAD placement between May 2005 and May 2011 was performed to collect demographics, cardiac diagnoses, details of VADs, and transthoracic and transesophageal echocardiographic findings from the time of initial diagnosis until VAD explantation. RESULTS: The study included 32 patients (median age, 3 years; age range, 20 days to 16 years; median weight, 12.3 kg; weight range, 3.5-60 kg), 20 with left ventricular assist devices and 12 with biventricular assist devices. Diagnoses included dilated cardiomyopathy or myocarditis (n = 27) and congenital heart disease (n = 5). The median duration of support was 12 days (range, 1-141 days). Patients with decreased right ventricular function were 8 times more likely to undergo biventricular assist device placement compared with those with normal right ventricular function (P = .026). Pre-VAD intracardiac shunts were identified in 11 patients and intracardiac thrombus in one patient. Cardiac chamber dimensions and mitral insufficiency were significantly reduced after VAD implantation. Postimplantation pericardial effusions were recognized in 16 patients and pericardial hematomas in 12 patients. CONCLUSIONS: Echocardiography is invaluable in the management of pediatric patients receiving VADs. It is helpful in pre-VAD assessment, guiding intraoperative device placement, recognizing VAD dysfunction, and identifying postimplantation complications.

Conal septal morphometrics can identify higher risk neonates with tetralogy of Fallot.

BACKGROUND: Some neonates with tetralogy of Fallot (TOF) have rapid progression of right ventricular outflow tract obstruction, requiring early repair irrespective of Doppler gradient as measured in the neonatal period. The aim of this study was to test the hypothesis that infundibular morphology in neonates with TOF is associated with the occurrence of hypercyanotic spells and need for neonatal surgery. METHODS: Fifty patients with TOF undergoing surgical repair from 2003 to 2009 were studied. Neonatal echocardiograms were retrospectively analyzed to measure conal septal angle (the angle between the conal septum and the horizontal plane passing through the center of the aortic valve in the parasternal short-axis view, with a larger angle denoting more anterocephalad deviation of conal septum), conal septal thickness and length, the degree of aortic dextroposition, and sizes and Z scores of the pulmonary annulus and the main and branch pulmonary arteries. Outcomes included the occurrence of hypercyanotic spells and the need for neonatal surgery. RESULTS: The median age at first echocardiogram was 2 days (range, 0-12 days). The median age at surgery was 94 days (range, 5-282 days); hypercyanotic spells occurred in 17 patients (34%), and nine (18%) underwent neonatal repair. The presence of a wider conal septal angle was significantly associated with the occurrence of hypercyanotic spells (59 ± 21° vs 48 ± 13°, P = .023) and the need for neonatal surgery (67 ± 13° vs 48 ± 16°, P = .004). The positive and negative predictive values of hypercyanotic spells for conal septal angles ≥60° were 64% and 78%, respectively. Importantly, Doppler right ventricular outflow tract gradient at initial echocardiography, degree of aortic dextroposition, and pulmonary or aortic valve size were not associated with these outcomes. CONCLUSIONS: A wider conal septal angle is associated with the occurrence of hypercyanotic spells and the need for neonatal surgery.

Early detection of cardiac dysfunction by strain and strain rate imaging in children and young adults with marfan syndrome.

OBJECTIVES: Strain and strain rate imaging have been reported to detect cardiac dysfunction more accurately than conventional methods in adults with Marfan syndrome, but their utility has not been proven in younger patients. We sought to determine whether strain and strain rate imaging would allow early detection of cardiac dysfunction in children and young adults with Marfan syndrome. METHODS: Unoperated patients (<30 years) with Marfan syndrome and healthy control participants were prospectively enrolled. Patients with greater than mild mitral or aortic insufficiency were excluded. Left ventricular systolic function was assessed by standard M-mode imaging. The strain and strain rate values were obtained from apical 4-chamber (longitudinal) and parasternal short-axis views at basal and midventricular levels (radial and circumferential). Data from the two groups were compared by a t test. RESULTS: Sixteen patients with Marfan syndrome (mean age ± SD, 14.4 ± 6.4 years; range, 5.8-28.9 years) and 26 controls (mean age, 12.4 + 4.4 year; range, 4.1-18.1 years) were enrolled. Demographics and left ventricular end-diastolic dimensions were similar between the patients with Marfan syndrome and the controls. The M-mode-derived shortening fraction was significantly lower in the patients with Marfan syndrome compared to the controls, even though the values were within normal limits. The patients with Marfan syndrome had lower regional radial and circumferential strain rates, but there were no significant difference in strain between the groups. CONCLUSIONS: Strain rate imaging may be useful in detection of subclinical changes in cardiac function in patients with Marfan syndrome when conventional echocardiographic parameters are within normal limits. These findings may be clinically important and warrant closer follow-up of these patients to monitor for cardiac dysfunction.

MR imaging and CT evaluation of congenital pulmonary vein abnormalities in neonates and infants.

Magnetic resonance (MR) imaging and computed tomography (CT) are increasingly being used in diagnosis and follow-up of congenital pulmonary vein anomalies in neonates and infants. Such anomalies include total or partial anomalous pulmonary venous return, sinus venosus defect, malposition of the septum primum, cor triatriatum, pulmonary vein atresia or stenosis, and abnormal number or course of the pulmonary veins. MR imaging provides a wealth of anatomic and functional data that are valuable in case management and planning intervention. Gadolinium-enhanced MR angiography is the mainstay of anatomic evaluation. Ventricular volumetry with two-dimensional steady-state free-precession sequences and flow analysis with cine phase-contrast imaging provide physiologic information that may be used to calculate the degree of right heart enlargement and the shunt fraction, allowing the cardiologist to determine the functional importance of the lesion. CT provides superior spatial resolution and short imaging times but at the expense of exposure to ionizing radiation.

Noninvasive estimation of diastolic pulmonary artery pressure by Doppler analysis of tricuspid regurgitation velocity in pediatric patients.

PURPOSE: Diastolic pulmonary artery pressure (dPAP) is equal to right ventricular pressure at the time of pulmonary valve opening. We studied the accuracy of dPAP estimated from Doppler profile of tricuspid regurgitation (TR) jet in pediatric patients. METHODS: Echocardiograms were prospectively performed on consecutive pediatric heart transplant recipients undergoing right-heart catheterization and endomyocardial biopsy. An estimate of dPAP was obtained by superimposing the pulmonary valve opening time, indexed to the electrocardiogram, onto the TR Doppler tracing. Echocardiographic estimates of dPAP from end-diastolic pulmonary regurgitation (PR) were obtained for comparison. Catheter-derived right atrial pressure was added to the Doppler gradient in both groups. Doppler estimates and catheter-derived measurements of dPAP were compared using Lin correlation and Bland-Altman analysis. RESULTS: Sixty-five catheterization studies were performed on 35 patients (20 males): median age at enrollment: 12.1 years (4 months to 18 years); median time: since transplant of 1.2 years (21 days to 16.1 years). Adequate TR signal was obtained in a significantly higher proportion of patients than an adequate PR signal (65% vs. 43%, respectively, P = .007). Median catheter-derived dPAP was 12 mm Hg (6-30 mm Hg) and right atrial pressure was 6 mm Hg (1-17 mm Hg). Median estimated dPAP from TR was 15 mm Hg (range: 7-29 mm Hg), with the Lin correlation coefficient of 0.74 (95% confidence interval [CI]: 0.6-0.87). Median estimate for dPAP from PR was 10 mm Hg (range: 2-25 mm Hg), with the Lin correlation coefficient of 0.74 (95% CI: 0.58-0.9). There was excellent interobserver agreement for dPAP from TR with the Lin correlation coefficient of 0.946 (95% CI: 0.803-0.986). CONCLUSION: Doppler estimation of dPAP from TR is a novel, reliable, noninvasive method and compares favorably with estimation from PR. Adequate TR signal for estimation of dPAP can be obtained more frequently in children than adequate PR signal, thereby increasing the proportion of patients in whom dPAP can be estimated noninvasively.

Myocardial tissue Doppler velocities in fetuses with hypoplastic left heart syndrome.

BACKGROUND: Tissue Doppler Imaging (TDI) is a sensitive index of myocardial function. Its role in the fetus has not been extensively evaluated. OBJECTIVE: To compare myocardial tissue Doppler velocities in fetuses with hypoplastic left heart syndrome (HLHS) to those of normal fetuses (matched for gestational age.) METHODS: Cross-sectional retrospective study conducted at 2 large perinatal centers (2003-2007). Fetuses with HLHS (n = 13) were compared with normal fetuses (n = 207) in 5 gestational age groups. TDI data included peak systolic (s'), peak early (e'), and late diastolic velocities (a'). Linear regression was used to compare TDI parameters in fetuses with HLHS to normal fetuses matched for gestational age. RESULTS: Fetuses with HLHS had significantly reduced lateral tricuspid annular e' as compared to normal fetuses. Both normal fetuses and those with HLHS had linear increase in TDI velocities with advancing gestational age. CONCLUSIONS: TDI velocities are abnormal in fetuses with HLHS. TDI can be useful in serial follow-up of cardiac function in fetuses with HLHS.