Successful treatment of dumping syndrome with diazoxide in an infant with hypoplastic left heart syndrome.

Summary: Dumping syndrome is a rare but potentially serious condition that causes inappropriate postprandial hyperinsulinemia leading to hypoglycemia in children following gastrointestinal surgeries. While dietary modifications are often the first line of treatment, severe cases may require pharmacological intervention to prevent severe hypoglycemia. We present a case of successful treatment of dumping syndrome with diazoxide. A 2-month-old infant with left hypoplastic heart syndrome who underwent single ventricle palliation pathway and developed feeding intolerance that required Nissen fundoplication. Postprandial hypoglycemia was detected following the procedure, with glucose level down to 12 mg/dL, and the diagnosis of dumping syndrome was established. The patient was successfully managed with diazoxide, which effectively resolved postprandial hypoglycemia without any major adverse events. The patient was eventfully weaned off the medication at the age of 5 months. This case highlights the potential role of diazoxide in the management of pediatric patients with postprandial hyperinsulinemic hypoglycemia secondary to dumping syndrome. Learning points: Dumping syndrome is a possible complication of gastrointestinal surgeries and should be suspected in children with abnormal glucose levels. Postprandial hyperglycemia should be monitored closely for significant subsequent hypoglycemia. Diazoxide might be considered as part of the treatment plan for dumping syndrome.

Infective intracardiac lesion in the setting of Mycobacterium franklinii bacteremia identified by cell-free DNA sequencing in a child with acute lymphoblastic leukemia: A potential new foe in intracardiac infections.

Mycobacterium franklinii (Mfra) is a recently identified member of the Mycobacterium chelonae-abscessus complex (MCAC), a rapidly growing, acid-fast bacilli that have the potential to cause invasive human infections. Identification of Mfra is crucial for selecting the appropriate antimicrobial therapy, as Mfra displays a unique susceptibility profile compared to other MCAC members. The literature on Mfra is limited, with a few studies focusing on respiratory and skin infections. To our knowledge, we describe the first reported case of cardiac involvement associated with Mfra bacteremia in a patient with acute lymphoblastic leukemia. The isolation of Mfra through a next-generation sequencing test allowed for prompt identification and subsequent implementation of tailored antimicrobial agents, ultimately resulting in positive clinical outcomes. This case also emphasizes the significance of next-generation testing in managing immunocompromised patients with persistent fever.

Long-Term Outcomes After Melody Transcatheter Pulmonary Valve Replacement in the US Investigational Device Exemption Trial.

BACKGROUND: The Melody valve was developed to extend the useful life of previously implanted right ventricular outflow tract (RVOT) conduits or bioprosthetic pulmonary valves, while preserving RV function and reducing the lifetime burden of surgery for patients with complex congenital heart disease. METHODS: Enrollment for the US Investigational Device Exemption study of the Melody valve began in 2007. Extended follow-up was completed in 2020. The primary outcome was freedom from transcatheter pulmonary valve (TPV) dysfunction (freedom from reoperation, reintervention, moderate or severe pulmonary regurgitation, and/or mean RVOT gradient >40 mm Hg). Secondary end points included stent fracture, catheter reintervention, surgical conduit replacement, and death. RESULTS: One hundred seventy-one subjects with RVOT conduit or bioprosthetic pulmonary valve dysfunction were enrolled. One hundred fifty underwent Melody TPV replacement. Median age was 19 years (Q1-Q3: 15-26). Median discharge mean RVOT Doppler gradient was 17 mm Hg (Q1-Q3: 12-22). The 149 patients implanted >24 hours were followed for a median of 8.4 years (Q1-Q3: 5.4-10.1). At 10 years, estimated freedom from mortality was 90%, from reoperation 79%, and from any reintervention 60%. Ten-year freedom from TPV dysfunction was 53% and was significantly shorter in children than in adults. Estimated freedom from TPV-related endocarditis was 81% at 10 years (95% CI, 69%-89%), with an annualized rate of 2.0% per patient-year. CONCLUSIONS: Ten-year outcomes from the Melody Investigational Device Exemption trial affirm the benefits of Melody TPV replacement in the lifetime management of patients with RVOT conduits and bioprosthetic pulmonary valves by providing sustained symptomatic and hemodynamic improvement in the majority of patients. Registration: URL: https://www.clinicaltrials.gov; Unique identifier: NCT00740870.

Comparison of the investigational device exemption and post-approval trials of the Melody transcatheter pulmonary valve.

OBJECTIVE: We compared 5-year outcomes of transcatheter pulmonary valve (TPV) replacement with the Melody TPV in the post-approval study (PAS) and the investigational device exemption (IDE) trial. BACKGROUND: As a condition of approval of the Melody TPV after the IDE trial, the Food and Drug Administration required that a PAS be conducted to evaluate outcomes of TPV replacement in a "real-world" environment. The 5-year outcomes of the PAS have not been published, and the IDE and PAS trials have not been compared. METHODS: The cohorts comprised all patients catheterized and implanted at 5 IDE sites and 10 PAS sites. Differences in trial protocols were detailed. Time-related outcomes and valve-related adverse events were compared between the two trials with Kaplan-Meier curves and log-rank testing. RESULTS: 167 patients (median age, 19 years) were catheterized and 150 underwent TPV replacement in the IDE trial; 121 were catheterized (median age, 17 years) and 100 implanted in the PAS. Freedom from hemodynamic dysfunction (p = .61) or any reintervention (p = .74) over time did not differ between trials. Freedom from stent fracture (p = .003) and transcatheter reintervention (p = .010) were longer in PAS, whereas freedom from explant (p = .020) and TPV endocarditis (p = .007) were shorter. Clinically important adverse events (AEs) were reported in 14% of PAS and 7.2% of IDE patients (p = .056); the incidence of any particular event was low in both. CONCLUSIONS: Hemodynamic and time-related outcomes in the PAS and IDE trials were generally similar, confirming the effectiveness of the Melody TPV with real-world providers. There were few significant complications and limited power to identify important differences in AEs. The lack of major differences in outcomes between the two studies questions the usefulness of mandated costly post-approval studies as part of the regulatory process for Class III medical devices.

Risk Factors for Longer Hospital Stay Following the Fontan Operation.

OBJECTIVES: Children with functional single ventricle undergoing the Fontan operation consume considerable resources. The purpose of this study is to evaluate pre- and intraoperative risk factors for longer hospital stay and to describe the perioperative course at a single institution over a 15-year period. DESIGN: Retrospective cohort study. SETTING: A single pediatric cardiac ICU. PATIENTS: All consecutive patients undergoing a first-time Fontan operation from 2000 to 2014. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Prolonged length of stay was defined as hospital stay greater than 75 percentile at our institution after surgery. Of 218 patients who met inclusion criteria, median length of stay was 10 days (interquartile range, 8-14 d); prolonged length of stay was defined greater than or equal to 15 days. Independent pre- and intraoperative risk factors for prolonged length of stay included higher hemoglobin (odds ratio, 1.29; p = 0.003), higher mean pulmonary artery pressure (odds ratio, 1.14; p = 0.037), and lower aortic saturation (odds ratio, 0.92; p = 0.008) in the entire group. When patients with hepatic vein inclusion (following previous Kawashima) were excluded, higher hemoglobin (odds ratio, 1.24; p = 0.027), lower aortic saturation (odds ratio, 0.92; p = 0.017), and placement of a fenestration (odds ratio, 2.438; p = 0.021) were associated with prolonged length of stay. Fifty-eight patients (26.6%) had major complications defined by Pediatric Cardiac Critical Care Consortium. Placement of a fenestration (odds ratio, 2.297; p = 0.014) and longer aortic cross-clamp time (odds ratio, 1.015; p = 0.003) were independently associated with Pediatric Cardiac Critical Care Consortium major complications. CONCLUSIONS: In this series, 75% of patients had a postoperative length of stay less than or equal to 2 weeks. Preoperative factors suggesting worse hypoxemia/decreased pulmonary blood flow (higher hemoglobin and lower oxygen saturation) and increased pulmonary artery pressure were associated with prolonged length of stay. These findings may help risk stratify this complex patient population, provide more accurate family counseling, and provide preliminary data for changes in preoperative timing of the Fontan and/or changes to postoperative management strategies for those at high risk for increased ICU morbidity.

CRISP: Catheterization RISk score for Pediatrics: A Report from the Congenital Cardiac Interventional Study Consortium (CCISC).

OBJECTIVES: We sought to develop a scoring system that predicts the risk of serious adverse events (SAE's) for individual pediatric patients undergoing cardiac catheterization procedures. BACKGROUND: Systematic assessment of risk of SAE in pediatric catheterization can be challenging in view of a wide variation in procedure and patient complexity as well as rapidly evolving technology. METHODS: A 10 component scoring system was originally developed based on expert consensus and review of the existing literature. Data from an international multi-institutional catheterization registry (CCISC) between 2008 and 2013 were used to validate this scoring system. In addition we used multivariate methods to further refine the original risk score to improve its predictive power of SAE's. RESULTS: Univariate analysis confirmed the strong correlation of each of the 10 components of the original risk score with SAE attributed to a pediatric cardiac catheterization (P < 0.001 for all variables). Multivariate analysis resulted in a modified risk score (CRISP) that corresponds to an increase in value of area under a receiver operating characteristic curve (AUC) from 0.715 to 0.741. CONCLUSION: The CRISP score predicts risk of occurrence of an SAE for individual patients undergoing pediatric cardiac catheterization procedures.

CRISP: catheterization RISk score for pediatrics: a report from the congenital cardiac interventional study consortium (CCISC)

OBJECTIVES:We sought to develop a scoring system that predicts the risk of serious adverse events (SAE's) for individual pediatric patients undergoing cardiac catheterization procedures. BACKGROUND: Systematic assessment of risk of SAE in pediatric catheterization can be challenging in view of a wide variation in procedure and patient complexity as well as rapidly evolving technology. METHODS: A 10 component scoring system was originally developed based on expert consensus and review of the existing literature. Data from an international multi-institutional catheterization registry (CCISC) between 2008 and 2013 were used to validate this scoring system. In addition we used multivariate methods to further refine the original risk score to improve its predictive power of SAE's. RESULTS: Univariate analysis confirmed the strong correlation of each of the 10 components of the original risk score with SAE attributed to a pediatric cardiac catheterization (P < 0.001 for all variables). Multivariate analysis resulted in a modified risk score (CRISP) that corresponds to an increase in value of area under a receiver operating characteristic curve (AUC) from 0.715 to 0.741. CONCLUSION: The CRISP score predicts risk of occurrence of an SAE for individual patients undergoing pediatric cardiac catheterization procedures.

Balloon angioplasty and stent implantation performed through systemic-to-pulmonary artery shunts in infants and neonates.

Branch pulmonary artery stenosis (BPAS) in the setting of systemic-pulmonary artery shunts (SPS) may result in significant sequelae. Limited information exists regarding the safety and efficacy of pulmonary artery balloon angioplasty and stent implantation via SPS in neonates and infants. This study aimed to examine the feasibility, safety, and efficacy of balloon angioplasty/stent implantation for BPAS performed via SPS in neonates and infants. A single-center retrospective analysis of all patients weighing 10 kg or less who underwent angioplasty for BPAS via SPS was performed. Systemic oxygen saturations and vessel diameter before, during, and after the procedure were compared. Between July 1996 and February 2008, 15 patients underwent 20 catheterizations for the treatment of 27 BPAS via SPS. The patients had a mean weight of 5.6 kg (range, 2.6-10 kg) and a mean age of 7.6 months (range, 7 days to 33 months). The SPS diameter ranged between 2 and 5 mm (median, 3.5 mm). Angioplasty was performed for all 27 lesions, and an additional stent was placed in 5 of these. The average lesion diameter increased from 2.3 ± 1.5 to 4.7 ± 1.7 mm (p < 0.05), and 25 (93 %) of the 27 lesions met the predetermined criteria for success. Systemic oxygen saturation increased from 73 ± 9.5 % to 82 ± 6.8 % immediately after intervention and was 83 ± 7.9 % at discharge (p < 0.05). There were no instances of shunt thrombosis. Two patients experienced transient hypotension during the procedure. No procedural deaths occurred. The study findings suggest that balloon angioplasty or stent implantation performed via SPS appears to be safe and effective treatment for BPAS in neonates and infants.

The use of three-dimensional rotational angiography to assess the pulmonary circulation following cavo-pulmonary connection in patients with single ventricle.

BACKGROUND: Bi-plane angiography is the gold standard for imaging the pulmonary arteries (PAs) after cavo-pulmonary connection (CPC). Three-dimensional rotational angiography (3DRA) is emerging technology that we hypothesized may enhance diagnostic capabilities in this patient group. METHODS: Retrospective review of patients who underwent 3DRA to assess the PAs after CPC. 3DRA images were examined for diagnostic quality and compared, both qualitatively and quantitatively with corresponding 2D images. RESULTS: Between 1/2010 and 12/2010, 37 3DRAs were obtained in 32 pts with prior CPC. Median age and weight were 4.3 years (0.3-19) and 15.7 kg (4.3-114). Previous surgery included: bi-directional Glenn (16), bilateral bi-directional Glenn (2), and Fontan (14). Thirty-three (89%) acquisitions in 28 pts were of diagnostic quality. In 19/28 pts (68%) 3DRA provided significant additional information pertaining to 22 lesions when compared with bi-plane angiography. These included delineating the mechanism of PA stenosis in 13 (aortic compression: 7, PA torsion: 6), demonstrating a discrete PA stenosis not seen or under appreciated on corresponding 2D images in 9. This additional information led to the performance of 9 interventions (stent implantation 8, angioplasty 1). Corresponding 2D and 3DRA images of the right and left PA were compared using quantitative analysis with a correlation coefficient of 0.94 (P < 0.001) and 0.97 (P < 0.001), for the right and left PAs respectively. CONCLUSIONS: 3DRA can be performed safely and effectively following CPC and may be more sensitive in diagnosing PA stenoses than 2D angiography. Quantitative measurements of PA diameter using 3DRA correlates well with 2D angiography.

The Fontan operation: the pursuit of associated lesions and cumulative trauma.

Our programmatic approach to the Fontan operation has evolved to include using an extracardiac conduit with aggressive presumptive treatment of associated lesions either in the catheterization laboratory or the operating room. Fenestration is used selectively based on hemodynamics, anatomy, and presence of associated lesions. We reviewed our experience to determine the effectiveness and outcome of this strategy and to assess the cumulative trauma to the patients. The records of 137 consecutive patients who underwent Fontan at Miami Children's Hospital from 1995 to 2008 were reviewed. At mean follow up of 5.76 years, freedom from death or transplantation is 94.2% (129/137). Median age at operation was 4.6 years. Longer length of stay correlated with older operative age (P = 0.0056). Pacemakers were implanted in 11.7% (16/137). Additional (not pre-Glenn or pre-Fontan) interventional catheterizations were performed in 51.8% (71/137). Additional operations were done in 10.2% (14/137). No patient has required replacement or revision of the extracardiac conduit. Our current approach to the Fontan operation provides acceptable midterm results. The pursuit of residual lesions results in a significant number of additional interventional catheterizations and operative procedures but might have an important influence on long-term survival after the Fontan procedure.

Physiologic limitation of omega exists in patients after the Norwood palliation and is underestimated by the arterial venous oxygen saturation difference.

BACKGROUND: Survival after the Norwood palliation (NW) is dependent on maximizing systemic oxygen delivery. Omega (Omega) is used to express the excess of oxygen delivery to oxygen consumption. We hypothesized that an intrinsic deficiency in the ability to maximize the relationship between oxygen delivery (DO(2)) and oxygen consumption (VO(2)) is present in infants after NW and is underestimated by arterial venous oxygen saturation difference (a-vO(2)). METHODS: Simultaneous arterial and systemic venous blood gas data were prospectively collected for the first 24 hours after surgery in 10 neonates after NW and 32 infants after biventricular repair (BV). Blood gas data were compared between groups. These data were compared with a group of 11 infants with hypoplastic left heart syndrome (HLHS) status post NW who were undergoing routine hemodynamic evaluation in the cardiac catheterization lab prior to a bidirectional Glenn operation (pre-Glenn). RESULTS: The mean systemic arterial oxygen saturation was 77% for NW, 83% for pre-Glenn, and 97% for biventricular repair. NW group had a mean systemic venous oxygenation saturation (SvO(2)) of 47% (range 12-67%) vs. 67% (range 29-84%) in the BV group (P = .001). Pre-Glenn patients had a SvO(2) of 46 (not significant [NS] vs. NW, P = .001 vs. BV). In NW, a-vO(2) mean was 32 (range 15-63) compared with mean 32 (range 16-71) in BV group (NS). Pre-Glenn a-vO(2) mean was 33.6 (NS vs. NW or BV). Omega for NW group was 2.6 (range 1.1-4.2) and 3.4 (1.4-6.25) in BV group (P = .001). Omega for pre-Glenn was 2.5 (range 1.7-3.8) (NS vs. NW, P = .001 vs. BV). CONCLUSION: Omega is lower for patients in the immediate postoperative period after NW and prior to their bidirectional Glenn operation than in patients after biventricular repair. This places NW patients closer to the critical point of DO(2) and therefore at greater risk for hemodynamic compromise than BV patients. The a-vO(2) difference underestimates this risk.

Goal-directed medical therapy and point-of-care testing improve outcomes after congenital heart surgery.

OBJECTIVE: A goal-directed therapy algorithm based on serial lactate values obtained from a point-of-care testing device was utilized in an attempt to reduce the mortality of patients after congenital heart surgery. DESIGN: Prospective study of patients undergoing surgery utilizing a goal-directed therapy algorithm in the postoperative period. The results of this group are compared with a historical cohort. Operative risk was determined using the RACHS-1 scoring system. SETTING: A 12-bed cardiac intensive care unit (ICU) in a pediatric hospital. PATIENTS: Patients undergoing surgery from July 2001 through September 2003 (group B, n=710) were compared to cohorts from June 1995 through June 2001 (group A, n=1,656). Group B patients were smaller and younger (median weight 6.2 vs 8 kg, p<0.001; median age 184 vs 327 days, p=0.004). INTERVENTIONS: Beginning in July 2001, blood lactate measurements were performed serially for 24 h after heart surgery. Based on lactate values and trends, therapy was amended. MEASUREMENTS AND RESULTS: Mortality was lower for group B (1.8 vs 3.7%, p=0.02). A reduction in mortality between group B and group A was noted in neonates (3.4 vs 12%, p=0.02), but not in older patients. Group B patients undergoing higher risk operations (Risk Adjustment for Congenital Heart Surgery-1 [RACHS-1] categories 3-6) had a significant reduction in mortality when compared to group A (3 vs 9%, p=0.006), no difference was noted in patients undergoing lower risk operations (RACHS-1 categories 1 and 2). CONCLUSIONS: The combination of goal-directed therapy and point-of-care testing was associated with a marked decrease in mortality for patients undergoing congenital heart surgery. Improvement was greatest in the highest risk patients.