A Simplified Staged Left Ventricular Recruitment Strategy to Achieve Biventricular Repair in a Neonate With Hypoplastic Left Heart Variant.

Hypoplastic left heart complex presents a diverse spectrum of variants. Traditional management has been dichotomous, involving either single ventricle palliation or high-risk biventricular repair. Surgical approaches to achieve biventricular repair in children with borderline left ventricle are continually evolving. One such method is the staged left ventricular recruitment (SLVR) strategy. Here, we illustrate a unique surgical approach to SLVR, achieving biventricular repair over 22 months in a neonate with hypoplastic left heart variant.

Procedural closure of the patent ductus arteriosus in preterm infants: a clinical practice guideline.

IMPORTANCE: Transcatheter closure of the patent ductus arteriosus (PDA) is being increasingly adopted as an alternative to surgical PDA closure in preterm infants. OBJECTIVE: To develop rigorous clinical practice guideline recommendations on procedural PDA closure in preterm infants. METHODS: The principles of the GRADE (Grading of Recommendations Assessment, Development and Evaluation) Evidence-to-Decision (EtD) framework were used to develop the guideline recommendations. An e-Delphi survey of 45 experts was conducted and recommendations that reached ≥75% agreement were accepted as consensus. MAIN RECOMMENDATIONS: Procedural PDA closure may be considered in extremely preterm infants (<28 weeks gestational age) requiring invasive mechanical ventilation >10 postnatal days and confirmed to have a large hemodynamically significant PDA, at centers with high local rates of death and/or bronchopulmonary dysplasia (conditional recommendation). If sufficient institutional expertise is available and patient characteristics are suitable, transcatheter PDA closure may be considered as the preferred approach over PDA ligation (conditional recommendation).

Rescue Sternotomy in a 540†g Neonate for Right Atrial Perforation During Percutaneous Ductal Device Occlusion.

Percutaneous ductal device closure in neonates is gaining popularity. Cardiac perforation is a rare but catastrophic complication that can occur during this procedure. Surgical options to salvage this situation are limited in extremely low-weight babies. In this report, we describe one such case managed successfully and offer some suggestions to achieve a successful outcome.

Percutaneous Closure of the Patent Ductus Arteriosus in Infants ≤2 kg: IMPACT Registry Insights.

OBJECTIVES: Percutaneous patent ductus arteriosus (PDA) closure is becoming the standard of care for definitive closure in progressively smaller and younger neonates. The objective of this study was to assess safety and feasibility of percutaneous PDA closure in patients ≤2 kg. METHODS: This was a cohort study using the IMPACT Registry (Improving Pediatric and Adult Congenital Treatments) from the American College of Cardiology Foundation's National Cardiovascular Data Registry. Patients who were ≤2 kg at the time of percutaneous PDA closure were included. The primary outcome was the composite of technical failure and/or major adverse event. RESULTS: A total of 1587 attempted PDA closures were included, with a 3% incidence of technical failure and 5.5% incidence of the composite outcome. Major adverse events were observed in 3.8% of the patients; the most common events were device embolization requiring retrieval and unplanned cardiac or vascular surgery in 1.3% and 1.3% of cases, respectively. The incidence of the composite outcome was associated with the need for arterial access (P < .001) as well as annual hospital volume of percutaneous PDA closures in infants ≤2 kg (P = .001). The incidence of the composite outcome has decreased overtime, whereas median weight at the time of procedure has also diminished. CONCLUSIONS: Percutaneous PDA closure appears to be safe and feasible procedures in infants ≤2 kg. The incidence of major adverse events has continued to decline over the years and seems to have a strong correlation with individual center case volumes and expertise.

Percutaneous Stage 1 Palliation for Hypoplastic Left Heart Syndrome.

Surgical palliation for hypoplastic left heart syndrome still carries significant morbidity and mortality in neonates. We previously described a percutaneous stage 1 palliation (PS1P) in a swine experiment. Here we report the human application of the PS1P for hypoplastic left heart syndrome in the United States. The procedure is performed through a 4F sheath in the femoral vein. Bilateral pulmonary flow restrictors are implanted in the proximal branch pulmonary arteries and a stent within the ductus arteriosus. PS1P could postpone surgical repair beyond the neonatal period. It offers a simple, less invasive alternative to currently available operations for newborns with HLHS.

Transcatheter Pulmonary Valve Replacement With the Sapien Prosthesis.

BACKGROUND: There are limited published data focused on outcomes of transcatheter pulmonary valve replacement (TPVR) with either a Sapien XT or Sapien 3 (S3) valve. OBJECTIVES: This study sought to report short-term outcomes in a large cohort of patients who underwent TPVR with either a Sapien XT or S3 valve. METHODS: Data were entered retrospectively into a multicenter registry for patients who underwent attempted TPVR with a Sapien XT or S3 valve. Patient-related, procedural, and short-term outcomes data were characterized overall and according to type of right ventricular outflow tract (RVOT) anatomy. RESULTS: Twenty-three centers enrolled a total of 774 patients: 397 (51%) with a native/patched RVOT; 183 (24%) with a conduit; and 194 (25%) with a bioprosthetic valve. The S3 was used in 78% of patients, and the XT was used in 22%, with most patients receiving a 29-mm (39%) or 26-mm (34%) valve. The implant was technically successful in 754 (97.4%) patients. Serious adverse events were reported in 67 patients (10%), with no difference between RVOT anatomy groups. Fourteen patients underwent urgent surgery. Nine patients had a second valve implanted. Among patients with available data, tricuspid valve injury was documented in 11 (1.7%), and 9 others (1.3%) had new moderate or severe regurgitation 2 grades higher than pre-implantation, for 20 (3.0%) total patients with tricuspid valve complications. Valve function at discharge was excellent in most patients, but 58 (8.5%) had moderate or greater pulmonary regurgitation or maximum Doppler gradients >40 mm Hg. During limited follow-up (n = 349; median: 12 months), 9 patients were diagnosed with endocarditis, and 17 additional patients underwent surgical valve replacement or valve-in-valve TPVR. CONCLUSIONS: Acute outcomes after TPVR with balloon-expandable valves were generally excellent in all types of RVOT. Additional data and longer follow-up will be necessary to gain insight into these issues.

Experience of a single institution with femoral vein homograft as right ventricle to pulmonary artery conduit in stage 1 Norwood operation.

OBJECTIVE: Femoral vein homograft can be used be used as valved right ventricle to pulmonary artery conduit in the Norwood operation. We describe the results of this approach, including pulmonary artery growth and ventricular function. METHODS: A retrospective chart review of 24 consecutive neonates with hypoplastic left heart syndrome or complex single ventricle undergoing this approach between June 2012 and December 2017 was performed. Conduit valve competency and ventricular function were estimated using transthoracic echocardiogram, and pulmonary artery growth was measured using Nakata's index. Changes in ventricular function pre-Glenn and at latest follow-up were assessed by ordinal logistic regression with a general linear model to account for the correlation within the same patient over time. RESULTS: Median age at surgery was 4 days, and mean weight was 3 kg. There was no interstage mortality. A total of 21 patients have undergone Glenn operation, and 9 patients have completed the Fontan operation. None of the conduits developed thrombosis. Sixty-three percent of conduits remained competent in the first month, and 33% remained competent after 3 months of operation. Catheter interventions on conduits were necessary in 14 patients. Median Nakata index at pre-Glenn catheterization was 228 mm2/m2 (interquartile range, 107-341 mm2/m2). Right ventricular function was preserved in 83% of patients at a median follow-up of 34 (interquartile range, 10-46) months. CONCLUSIONS: Femoral vein homograft as a right ventricle to pulmonary artery conduit in the Norwood operation is safe and associated with good pulmonary artery growth and preserved ventricular function as assessed by subjective echocardiography. Catheter intervention of the conduit may be necessary.

Utility of the Medtronic microvascular plug™ as a transcatheter implantable and explantable pulmonary artery flow restrictor in a swine model.

BACKGROUND: A surgical pulmonary artery band (PAB) is used to control excessive pulmonary blood flow for certain congenital heart diseases. Previous attempts have been made to develop a transcatheter, implantable pulmonary flow restrictor (PFR) without great success. We modified a microvascular plug (MVP) to be used as a PFR. The objectives of this study were to demonstrate feasibility of transcatheter implantation and retrieval of the modified MVP as a PFR, and compare PA growth while using the PFR versus PAB. METHODS AND RESULTS: The PFR was implanted in eight newborn piglets in bilateral branch pulmonary arteries (PAs). Immediately post-PFR implantation, the right ventricular systolic pressure increased from a median of 20-51 mmHg. Transcatheter retrieval of PFR was 100% successful at 3, 6, and 9 weeks and 50% at 12-weeks post-implant. A left PAB was placed via thoracotomy in four other newborn piglets. Debanding was performed 6-weeks later via balloon angioplasty. On follow-up, the proximal left PA diameters in the PFR and the PAB groups were similar (median 8 vs. 7.1 mm; p = 0.11); albeit the surgical band sites required repeat balloon angioplasty secondary to recurrent stenosis. By histopathology, there was grade II vessel injury in two pigs immediately post-retrieval of PFR that healed by 12 weeks. CONCLUSIONS: Transcatheter implantation and retrieval of the MVP as a PFR is feasible. PA growth is comparable to surgical PAB, which is likely to require reinterventions. The use of the MVP as a PFR in humans has to be trialed before recommending its routine use.

A comprehensive program for preterm infants with patent ductus arteriosus.

OBJECTIVES: Patent ductus arteriosus (PDA) is a common finding in preterm infants. A hemodynamically significant PDA may require intervention for closure. This article aims to describe a transcatheter PDA closure (TCPC) program for preterm infants and the components of a comprehensive outpatient follow-up strategy. SETTING: A multidisciplinary team approach including neonatology, cardiology, anesthesiology, medical transport team, pulmonology, cardiac surgery, neurodevelopmental specialist, nutrition, speech therapy, social work, research collaborators, and other health care specialists is integral to the dedicated care and promotion of wellness of extremely low birth weight (ELBW) infants. PATIENTS: To date, we have performed TCPC on 134 ELBW infants weighing <2 kg at the time of the procedure, 54 of whom were <1 kg with the smallest weighing 640 g with a median gestation age of 25 weeks (range 23-27 weeks). INTERVENTIONS: A comprehensive follow-up strategy with the creation of the Memphis PDA Clinic was implemented. OUTCOME MEASURES: Respiratory support, tolerance of enteral feeds, growth, and neurodevelopmental progress are indicators of favorable outcomes. RESULTS: TCPC has benefited ELBW infants with faster weaning off the ventilator, increase in enteral feedings, and somatic growth with the overall shortening of the hospital length of stay. The Memphis PDA Clinic has ensured optimal postdischarge follow-up to improve long-term outcomes. CONCLUSIONS: TCPC is a safe and effective alternative to manage ELBW infants with a hemodynamically significant PDA. Comprehensive follow-up after discharge provided in a multispecialty clinic developed specifically for this unique population has been successful in improving outcomes.

Echocardiographic guidance for transcatheter patent ductus arteriosus closure in extremely low birth weight infants.

Echocardiographic imaging provides real-time guidance during transcatheter patent ductus arteriosus (PDA) closure in extremely low birth weight (ELBW) infants. Transthoracic echocardiogram provides detailed assessment of the PDA and surrounding structures prior to, during, and after transcatheter closure. This article aims to review the different echocardiographic techniques and concepts utilized during transcatheter PDA closure in ELBW infants.

Practice variation in the management of patent ductus arteriosus in extremely low birth weight infants in the United States: Survey results among cardiologists and neonatologists.

BACKGROUND: Patent ductus arteriosus (PDA) is highly prevalent in extremely low birth weight (ELBW), preterm infants. There are diverse management approaches for the PDA in ELBW infants. The objectives of this research were to identify current PDA management practices among cardiologists and neonatologists in the United States, describe any significant differences in management, and describe areas where practices align. METHODS: A survey of 10 questions based on the management of PDA in ELBW infants was conducted among 100 prominent neonatologists from 74 centers and 103 prominent cardiologists from 75 centers. Among the cardiologists, approximately 50% were interventionists who perform transcatheter PDA closures (TCPC). Fisher's exact test was performed to compare practice variations among neonatologists and cardiologists. A potentially biased audience including a combination of health care providers belonging to cardiology, neonatology, and surgery were also surveyed during the International PDA Symposium. The results of this survey were not included for statistical comparison, due to this audience being potentially influenced by the Symposium. RESULTS: Statistically significant differences were identified between neonatologists and cardiologists regarding the impact of PDA closure on morbidity and mortality, with 80% cardiologists responding that it does vs 54% of neonatologists (P < 0.001), the need for PDA closure (P < .001), and the preferred method of PDA closure if indicated (P < .001). There was agreement between neonatologists and cardiologists on symptomatic therapy; however more neonatologists favored watchful waiting over intervention in contrast to more cardiologists favoring intervention over observation (77% vs 95%, P < .001). Survey responses also identified a need for further training and research on TCPC. CONCLUSION: Neonatologists and cardiologists have notable differences in managing PDA, and continued discussion across cardiology and neonatology has the potential to facilitate more of a consensus on best management practices. Further investigation is needed to identify outcomes in transcatheter PDA closure, particularly in ELBW infants.

Variability in radiation dose and image quality: A comparison across fluoroscopy-system vendors, generations of equipment and institutions.

OBJECTIVES: To evaluate differences in radiation dose and image quality across institutions, fluoroscope vendors and generations of fluoroscopes for pediatric cardiac catheterization. BACKGROUND: Increased recognition of the potentially harmful effects of ionizing radiation has spurred technological advances in fluoroscopes, as well as increased focus on optimizing fluoroscope performance. There is currently little understanding of variability in the dose-image quality relationship across institutions, fluoroscope vendor and/or generation of equipment. METHODS: We evaluated latest generation fluoroscopes from Phillips, Siemens, GE, and Toshiba, and an older generation Phillips fluoroscope (release date 2003) at three different institutions. Radiation dose was measured using an anthropomorphic dose-assessment phantom with effective dose in mSv estimated from Monte Carlo simulations. Image quality phantom images were scored on a 12-point scale by three blinded reviewers. RESULTS: Fluoroscope effective doses ranged from 0.04 to 0.14 mSv/1,000 pulses for fluoroscopy with associated composite image quality scores ranging from 8.0 ± 0.6 to 10.4 ± 1.3. For cineangiography, effective doses ranged from 0.17 to 0.57 mSv/1,000 frames with image quality scores ranging from 10.1 ± 0.3 to 11.1 ± 0.3. There was modest correlation between effective dose and image quality (r = 0.67, P = 0.006). The older generation fluoroscope delivered consistently higher doses than the newer generation systems (2.3- to 3.5-fold higher for fluoroscopy; 1.1- to 3.4-fold higher for cineangiography) without appreciable differences in image quality. CONCLUSION: Technological advances have markedly improved fluoroscope performance. Comparing latest generation systems across vendors and institutions, we found variability in the dose-IQ relationship and speculate that this reflects both equipment and institutional optimization practices.

Improved Outcomes in Management of Hypoplastic Left Heart Syndrome Associated With Congenital Diaphragmatic Hernia: an Algorithmic Approach.

Hypoplastic left heart syndrome (HLHS) is the second most common congenital heart disease associated with congenital diaphragmatic hernia (CDH). The reported survival rate of neonates with CDH and HLHS is only 1%-5%. We review our experience with CDH and HLHS and compare our outcomes with published literature. Retrospective review of all neonates with CDH and HLHS at our institution over a 10-year period was performed. The morphology of cardiac and diaphragm defects, clinical course, treatment strategies, and outcomes were reviewed, and an algorithmic approach was proposed. Five patients with CDH and HLHS were treated between 2006 and 2016. All had mitral stenosis with aortic stenosis. Four patients had a left-sided Bochdalek diaphragmatic hernia and 1 patient had a large bilateral Morgagni hernia. Two (2/4) of the Bochdalek hernias were associated with significant pulmonary hypoplasia and required patch closure of the CDH; both were palliated with percutaneous ductal stents and both died. Three patients underwent primary Norwood operation followed by repair of less severe CDH defect. All 3 patients are currently well and have survived bidirectional Glenn anastomosis; one patient is well after Fontan operation. Successful palliation of neonates with HLHS and associated CDH is possible in the current era. Outcome is determined primarily by the severity of the CDH and the degree of associated pulmonary hypoplasia. An algorithmic team approach is helpful in management of this difficult group of patients.

Acute and midterm results following perventricular device closure of muscular ventricular septal defects: A multicenter PICES investigation.

OBJECTIVES: To describe acute and mid-term results of hybrid perventricular device closure of muscular ventricular septal defects (mVSDs). BACKGROUND: Perventricular device closure of mVSDs can mitigate technical limitations of percutaneous closure and need for cardiopulmonary bypass or ventriculotomy with a surgical approach. METHODS: This is a multicenter retrospective cohort study of patients undergoing hybrid perventricular mVSD device closure from 1/2004 to 1/2014. Procedural details, adverse events, outcomes, and follow-up data were collected. Patients were divided into two groups: (1) simple (mVSD closure alone) and (2) complex (mVSD closure with concomitant cardiac surgery). RESULTS: Forty-seven patients (60% female) underwent perventricular mVSD device closure at a median age of 5.2 months (IQR 1.8-8.9) and weight of 5.1 kg (IQR 4.0-6.9). Procedural success was 91% [100% (n = 22) simple and 84% (n = 21/25) complex]. Adverse events occurred in 19% (9/47) [9% (2/22) simple and 28% (7/25) complex]. Hospital length of stay (LOS) was shorter in the simple vs. complex group (4 vs. 14 days, P < 0.01). At mid-term follow-up of 19.2 months (IQR 2.3-43) 90% of pts had complete mVSD closure; none developed late heart block, increased atrioventricular (AV) valve insufficiency or ventricular dysfunction. CONCLUSIONS: Perventricular device closure of simple mVSD was associated with a high rate of procedural success, few adverse events, and short hospital LOS. Procedural adverse events were associated with the presence of concomitant complex surgery. Residual mVSD, AV valve insufficiency, or ventricular dysfunction were uncommon at mid-term follow-up. © 2017 Wiley Periodicals, Inc.

Acute and long-term effects of endovascular debanding of pulmonary arteries in a swine model.

OBJECTIVES: The primary objective of this study was to demonstrate that pulmonary artery (PA) debanding via cardiac catheterization using balloon angioplasty is feasible and safe in swine. The secondary objectives were to determine the acute and long-term effects of this therapy. DESIGN: This is a chronic survival experimental study in newborn swine. BACKGROUND: PA bands are used in infants for transient palliation of congenital heart defects with excessive pulmonary blood flow. Although rare, if these defects should close spontaneously or become hemodynamically insignificant, a sternotomy and occasionally cardiopulmonary bypass may still be required for band removal. Alternatively, debanding could be accomplished through less invasive methods. INTERVENTIONS: The main pulmonary artery was banded in three piglets, and the left pulmonary artery in five piglets via mini-thoracotomy at a mean weight of 2.5 kg. Following a threefold increase in weight, the piglets underwent PA debanding via balloon angioplasty. Four piglets were sacrificed to evaluate the acute effects. The remainder were followed to evaluate long-term effects. Histopathology was performed on all piglets. OUTCOME MEASURES: Reintervention rates. Histopathologic consequences of high pressure balloon angioplasty used for PA debanding acutely and after reinterventions. RESULTS: Debanding was performed at a mean weight of 8.1 ± 2.23 kg. The median preintervention gradient across the band was 18 mm Hg. Debanding was successful in all piglets. The median postintervention gradient was 3.5 mm Hg. All piglets in the long-term model required re-interventions for recurrent stenosis at mean weights of 26 ± 1.6 and 61 ± 3.2 kg. Histopathology demonstrated vessel wall injury in only one piglet. CONCLUSIONS: Endovascular PA debanding can be safely achieved in a swine model. Angioplasty following debanding may be necessary for recurrent stenosis. This catheter-based therapy may provide a less-invasive alternative to surgery.

Surgical Management of Simultaneous Left Coronary Atresia and Anomalous Right Coronary Artery Origin.

A 9-year-old child presented with syncope during exercise. He received a diagnosis of congenital atresia of the left main coronary artery by angiography. He underwent successful coronary artery bypass grafting. On the third postoperative day, he experienced acute, precordial chest pain. An urgent computed tomographic scan showed an unrecognized anomalous origin of the right coronary artery (RCA) with a 1.5-cm intramural course. He was taken back to the operating room to undergo unroofing of the RCA. This case highlights the difficulty involved in making two rare diagnoses that can cause the same exact symptoms in a patient and the surgical challenges associated with it.

Femoral Vein Homograft as Right Ventricle to Pulmonary Artery Conduit in Stage 1 Norwood Operation.

BACKGROUND: The polytetrafluoroethylene tube used as right ventricle to pulmonary artery conduit in the stage 1 Norwood operation is associated with risks of suboptimal branch pulmonary artery growth, thrombosis, free insufficiency, and long-term right ventricular dysfunction. Our experience with use of valved femoral vein homograft as right ventricle to pulmonary artery conduit is described. METHODS: Between June 2012 and December 2015, 15 neonates with hypoplastic left heart syndrome or complex single ventricle underwent stage 1 Norwood operation with valved segment of femoral vein homograft as right ventricle to pulmonary artery conduit. The median age at surgery was 3 days and the mean weight was 3 kg. The size of the femoral vein homograft was 5 mm in 8 patients and 6 mm in 7 patients. RESULTS: There was no hospital or interstage mortality. Fourteen patients underwent Glenn operation, and 6 have undergone Fontan operation to date. The median Nakata index at pre-Glenn catheterization was 262 mm2/m2 (interquartile range: 121 to 422 mm2/m2). No patient had thrombosis of conduit. Most femoral vein conduits remained competent in the first month after stage 1 Norwood operation, although most became incompetent by 3 months. Catheter intervention on the conduit was necessary in 7 patients. Right ventricular function was preserved in most patients at follow-up. CONCLUSIONS: The use of femoral vein homograft as right ventricle to pulmonary artery conduit in the Norwood operation is safe and associated with good pulmonary artery growth and preserved ventricular function. Balloon dilation of the conduit may be necessary during the interstage period.

Clinical evaluation of a radio-protective cream for the hands of the pediatric interventional cardiologist.

OBJECTIVES: The aim of this study was to evaluate the effectiveness of UltraBLOX™ radiation attenuating hand cream during lengthy cardiac catheterization procedures in children. BACKGROUND: The hands of interventional cardiologists receive high doses of radiation due to their proximity to the X-ray beam. Radiation attenuating gloves have about a 26% attenuation rate, but reduce dexterity and tactile sensation. The UltraBLOX™ cream is a new FDA-approved X-ray attenuating cream that can be applied to the operator's hands for radio-protection. METHODS: Two nanoDot™ dosimeters were secured side by side on the dorsum of the operator's (n = 2) left hand close to the wrist. One dosimeter and the rest of the hand were covered with 0.2 mm layer of the cream. The other dosimeter was unshielded. Procedures were performed using 110 kVp fluoroscopy at 15 pulses/sec. The measurements were categorized into four groups dependent on the duration of the procedure. The patients in all four groups were well matched for age and size. RESULTS: Procedural and cumulative hand radiation doses were higher with longer procedural duration. The overall % attenuation by the cream was 39.7% (28.6-51.5) and was unaffected by the length of the procedure (median: 40.9% at 30 min and 41.4% at 180 min; P = 0.66) or the dose of radiation. The kappa statistic for interobserver agreement for good tactile sensitivity was 0.82. CONCLUSIONS: UltraBLOX™ cream provides a new option for radio-protection for the hands of interventional cardiologists without impairing tactile sensitivity. There was no decrease in attenuation up to 180 min. © 2016 Wiley Periodicals, Inc.

Successful Repair of Hypoplastic Left Heart Syndrome With Intact Atrial Septum, Congenital Diaphragm Hernia, and Anomalous Origin of Coronary Artery: Defying the Odds.

We report a case of an infant who was postnatally diagnosed with hypoplastic left heart syndrome and an intact atrial septum who underwent emergent atrial decompression followed by the Norwood operation. She was also found to have a congenital diaphragmatic hernia on the left side and a congenital eventration of the right diaphragm, both requiring surgical repair. She was later found to have an anomalous origin of the left circumflex coronary artery from the right pulmonary artery that was ligated at the time of the bilateral bidirectional Glenn operation. She is currently thriving at home, defying all odds.