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To determine if patients with congenital heart disease are undergoing laparoscopic surgery requiring abdominal insufflation and to compare the outcomes of these procedures with those who underwent an open surgical approach.This was a retrospective study using the National Inpatient Sample from 2006 to 2014. Individuals with congenital heart disease who underwent at least one of six selected surgical procedures (laparoscopic or open) were included in the study. Subgroup analysis was performed on patients with Fontan palliation.The primary outcome was to determine the frequency with which congenital heart disease patients undergo laparoscopic surgery requiring abdominal insufflation compared with open surgery. Secondary outcomes included all-cause in-hospital mortality and in-hospital length of stay. Of the 5,527 patients included, nearly half underwent laparoscopic surgery (46.3%), and 128 (2.3%) had single-ventricle circulation. All-cause mortality was significantly higher for those who underwent open surgery compared with the laparoscopic approach (3.6% v 0.9%; odds ratio [OR], 4.0 [2.6-6.3]; p0.0001). Subgroup analysis of patients with Fontal palliation older than five years showed 30 (42%) underwent laparoscopic surgery and there was no mortality difference between the laparoscopic and open approaches (OR, 1.4 [0.2-21.3], p = 0.8). Length of stay was significantly shorter for patients undergoing laparoscopic compared with open surgery (median three days [interquartile range, two-five] v six days [three-13], p0.0001).Individuals with congenital heart disease are being offered laparoscopic surgery that requires abdominal insufflation. All-cause mortality and length of stay were higher for patients who underwent open surgical operations.

Background: Alcohol consumption is associated with an increased risk of liver disease. There are limited studies on the epidemiology of alcohol use and its effects on Fontan-associated liver disease (FALD) in adulthood. Methods: In this single-center prospective cohort study, patients were enrolled from the Fontan clinic between November 2019 and November 2020, excluding those with chronic hepatitis C or B. Alcohol consumption was quantified by Alcohol Use Disorders Identification Test (AUDIT) questionnaire and a supplementary questionnaire. Participants were stratified into alcohol consumers and non-consumers. Alcohol consumption was correlated to the magnitude of fibrosis on liver biopsy and the varices, ascites, splenomegaly, and thrombocytopenia (VAST) score. Results: Forty-three patients (age 30 ​± ​6.5 years) were enrolled, and most were in NYHA FC 1 or 2. Twenty-six (60.5%) participants consumed alcohol regularly in the past year (twenty with low-risk consumption and six with hazardous consumption). Alcohol consumers were more likely to have better NYHA FC. Of those, half reported alcohol consumption for longer than one year before enrollment. Eleven (25.6%) participants reported underage drinking. After multivariable adjustment, male sex was associated with increased severity of liver fibrosis (OR 3.7 [1.0 to 13.6]). Alcohol consumption was not associated with liver fibrosis (OR 1.2 [0.3 to 4.9]) or VAST scores (OR 1.2 [0.01 to 2.2]). Conclusions: Alcohol consumption is prevalent among adults with the Fontan circulation but does not correlate with FALD. However, further studies are required to validate the results in cohorts with heavier alcohol consumption. Underage drinking was prevalent and warrants screening in pediatrics.

Objectives To report the first use of a balloon expandable bare metal stent for treating infant bronchomalacia. Background Infant bronchomalacia often requires prolonged mechanical ventilation and can be life-threatening. Effective treatment for severe infant bronchomalacia continues to be elusive. We present three cases of bronchial stenting for no-option or treatment refractory infant bronchomalacia. Methods Three consecutive cases of stenting to relieve conservative treatment refractory severe infant bronchomalacia were performed between February 2019 and December 2020. Initial diagnosis was confirmed with Computed Tomography (CT) angiography. Patients underwent rigid micro laryngoscopy, bronchoscopy, and flexible bronchoscopy to evaluate the airway. Initial conservative management strategies were pursued. Patients failing initial conservative management strategies were considered for rescue bronchial stenting. Results Our initial clinical experience with a coronary bare metal stent for these procedures has been favorable. The stent was easy to deploy with precision. We did not encounter stent embolization or migration. There was sufficient stent radial strength to relieve bronchomalacia without causing restenosis or erosion. There was no significant granulation tissue formation. In one patient, the stent was removed after 12 months of somatic growth; this was uneventful and bronchial patency was maintained. There were no complications in any of our patients regarding stent placement and reliability. Conclusion In cases of three infants with severe bronchomalacia, we found that bronchial stenting with the bare metal coronary stent was effective in relieving bronchial stenosis.

Background In the care of patients with congenital heart disease, percutaneous interventional treatments have supplanted many surgical approaches for simple lesions, such as atrial septal defect. By contrast, complex congenital heart defects continue to require open-heart surgery. In single-ventricle patients, a staged approach is employed, which requires multiple open-heart surgeries and significant attendant morbidity and mortality. A nonsurgical transcatheter alternative would be attractive. Objectives The authors sought to show the feasibility of catheter-only, closed-chest, large-vessel anastomosis (superior vena cava and pulmonary artery [PA] or bidirectional Glenn operation equivalent) in a patient. Methods In preclinical testing over a decade, the authors developed the techniques and technology needed for nonsurgical crossing from a donor (superior vena cava) to a recipient (PA) vessel and endovascular stent-based anastomosis of those blood vessels. The authors undertook this transcatheter approach for an adult with untreated congenital heart disease with severe cyanosis and significant surgical risk. They rehearsed the procedure step by step using contrast-enhanced cardiac computed tomography and a patient-specific 3-dimensional printed heart model. Results The authors describe a first-in-human, fully percutaneous superior cavopulmonary anastomosis (bidirectional Glenn operation equivalent). The patient, a 35-year-old woman, was homebound due to dyspnea and worsening cyanosis. She was diagnosed with functional single ventricle and very limited pulmonary blood flow. The heart team believed surgical palliation conferred high operative risk due to the patient’s complete condition. With the percutaneous procedure, the patient recovered uneventfully and remained improved clinically after 6 months. Conclusions This procedure may provide a viable alternative to one of the foundational open-heart surgeries currently performed to treat single-ventricle congenital heart disease.

Transcatheter electrosurgery is used for planned caval-aortic access and to facilitate transcatheter mitral and aortic valve implantation [(1,2)][1]. It has been used for percutaneous cavopulmonary shunt [(3)][2]. This technique can also be used for unplanned clinical rescue; we describe re

Pulmonary vein stenosis (PVS) in children is a progressive disease with poor outcomes. Surgical and catheter-based interventions can provide immediate relief of stenosis, but re-stenosis is common, often progressive, and can be fatal. We pursued serial balloon angioplasty (BA) to treat patients with recurrent PVS at our center. We performed a retrospective chart review and data analysis of all children (age

Neonatal myocardial infarction (MI), in the absence of congenital heart disease or cardiac surgery involving the coronaries, is a rare condition with associated high mortality. A cluster of neonatal myocardial infarction cases was observed, leading to an investigation of causes and contributors. We performed a single-center review of neonates >37 weeks between 2011 and 2017 to identify neonates with myocardial infarction. Neonates with prior cardiac surgery, congenital anomalies of the coronaries, or sepsis were excluded. Diagnosis of MI was based on ECG changes, elevated troponin, decreased function or regional wall abnormality, and abnormal coronary angiography. There were 4,583 admissions to the NICU without interval changes in referrals or the incidence of neonatal asphyxia. There were six cases of neonatal myocardial infarction. Of 2,155 admissions in the first three years, there were no cases. The incidence in the next three years was 1.3/1,000, 2.7/1,000, and 3.3/1,000. There was a significant difference (0.03, Fishers exact) between the first and second halves of the study period. All cases underwent catheterization, revealing coronary artery thrombosis requiring thrombolytic therapy. Additionally, obstetric providers were surveyed to determine changes in umbilical cord handling practices. Of 48 obstetric providers, 57% changed their practice of cord clamping since 2014: 13% reported cord milking then clamping; 47% delayed cord clamping for a few minutes; 28% delayed cord clamping until the cord stopped pulsating; and 13% immediate clamping. Only 40% reported routine documentation of cord clamping. In conclusion, there was an increased incidence of neonatal myocardial infarction due to coronary thrombosis. Early diagnosis and initiation of therapy of neonates with myocardial infarction can improve outcomes. A larger study cohort could lead to better understanding of risk factors and outcomes of myocardial infraction in neonates.

Right ventricular outflow tract (RVOT) conduit dysfunction is a limitation of the Ross procedure. Transcatheter pulmonary valve replacement (TPVR) could alter the impact of conduit dysfunction and the risk-benefit balance for the Ross procedure.Retrospective review of databases from 3 prospective Melody TPV (Medtronic Inc, Minneapolis, MN) trials.Among 358 patients who were catheterized with the intent to implant a Melody TPV for RVOT conduit stenosis or regurgitation (PR) as part of 3 prospective multicenter studies, 67 (19%) had a prior Ross procedure. Of these, 56 (84%) received a Melody valve; in 5 of the 11 patients who did not, the implant was aborted due to concern for coronary artery compression, and 1 implanted patient required emergent surgery for left coronary compression. The RVOT gradient decreased from a median 38 mm Hg to 13.5 mm Hg (p0.001). There was no or trivial PR in all but 4 patients, in whom it was mild. At a median follow-up of 4.0 years, 1 patient died from sepsis. Twelve patients underwent 14 transcatheter (n = 8) or surgical (n = 6) TPV reinterventions for obstruction with stent fracture (n = 9), endocarditis with conduit obstruction (n = 3), or reoperation (n = 2). Freedom from TPV explant was 89% ± 5% at 4 years. Among patients who did not undergo reintervention for obstruction, there was no change in RVOT gradient over time, and all but 1 patient had mild or less PR at last follow-up.The TPVR with the Melody valve provides acceptable early outcomes and durable valve function in the majority of Ross patients. Recurrent RVOT obstruction associated with stent fracture was the main reason for reintervention. Coronary compression is not uncommon in Ross patients and should be assessed prior to TPVR.

In 2007 we began a hybrid program for hypoplastic left heart syndrome (HLHS) variants to potentially improve outcome in high-risk patients. During implementation we offered both hybrid and Norwood approaches to all risk categories. The purpose of this study was to perform a comparative analysis of intermediate survival.Newborns were evaluated jointly for high-risk characteristics, including birth weight less than 2.5 kg, prematurity (especially35 weeks), central nervous system abnormalities, multiorgan failure, intact or severely restrictive atrial septum, severe ventricular dysfunction, and severe atrioventricular valve regurgitation. We prefer Norwood for standard risk and hybrid for high risk, but all groups crossed over into all treatment pathways resulting in the following 5 treatment groups: standard risk Norwood; high-risk Norwood; standard risk hybrid ductal stent (HDS); high-risk hybrid DS; and high-risk hybrid prostaglandin E1 (HPGE). We reviewed all consecutive patients from 2007 to 2012, obtained follow-up, and analyzed the results.Sixty-eight newborns presented (median 2.96 kg, 8 days); 29 (43%) were high and 39 (57%) were standard risk. There were 14 stage I hospital deaths strongly associated with risk: 3 of 39 standard (7.7%) and 11 of 29 high (38%, p = 0.002). Stage I discharge mortality was highest for high-risk Norwood and high-risk HPGE groups (p0.001). Actuarial survival up to 5 years demonstrated superior survival for Norwood versus hybrid (78.1% vs 56.4%, p = 0.0182). With risk stratification there was suboptimal survival for all 3 high-risk groups (p = 0.003); HDS fared better than HPGE but had higher birth weight (p0.001).While a risk-stratified approach for HLHS variant patients with selective use of hybrid palliation resulted in acceptable stage I mortality, the longer term mortality for high-risk patients remains higher than for standard risk regardless of treatment modality. Intrinsic patient risk factors (rather than treatment modality) likely determine long-term outcome in experienced centers. Our current high-risk approach has evolved to HPGE application with Norwood conversion whenever deemed medically possible.

Coronary artery fistula (CAF) is considered an embryologic persistence of primitive intra-trabecular spaces which allow the developing coronary artery to communicate with the other cardiac chambers or vascular structures. It is observed in 0.05–0.25% of coronary angiographic studies, most of which drain into a right heart chamber or into the pulmonary artery, while a congenital right coronary artery (RCA) into a left heart chamber is less frequent. 5 In this study, we describe an unusual case treated by closure device in the right coronary artery fistula to the left ventricle, and associated literature is reviewed. A 40-year-old female presented with chronic cough, otherwise asymptomatic. Echocardiogram revealed unusual flow into the LV with mild LV dilatation. A 64 multi-slice CT scan confirmed the presence of a huge right coronary opening with a fistula into the left ventricle. The decision was to close this fistula with device through the RCA into the left ventricle. The management of this unusually large fistula is described with focus on technical issues with device closure.

This review discusses the current safety issues related to U.S. Food and Drug Administration approved atrial septal defect devices and proposes a potential avenue to gather additional safety data including factors, which may be involved in device erosion.

Traditionally, all patients undergo cardiac catheterization before bidirectional cavopulmonary anastomosis (BCPA). The purpose of this study was to determine if preoperative catheterization is necessary when echocardiographic parameters appear favorable. A retrospective review was performed of all patients who underwent BCPA (n = 142) between February 1996 and May 2001. Echocardiographic criteria defining a favorable BCPA candidate included good ventricular function, moderate or less atrioventricular and semilunar valve regurgitation, absence of ventricular outflow tract obstruction, normal proximal branch pulmonary artery (PA) size, and low PA pressures estimated by PA band gradient or systemic PA shunt velocity. The median age at operation was 7 months (range: 2-11) and weight was 6.2 kg (range: 2.7-7.1). There were 73 unfavorable candidates. Patients with hypoplastic left heart syndrome (n = 23) and pulmonary atresia with intact septum (n = 15) predominated among the unfavorable group. All patients were catheterized. This provided additional information on PA pressures in 3 patients in the favorable group but did not defer operation or influence outcome (no mortality, prolonged pleural drainage, or longer intensive care department stay). All 3 patients are alive at a mean follow-up of 51 months. The 30-day mortality was 2% (4 of 151 patients), all in the unfavorable group. Overall, 20 patients (13%) required arterioplasty of PAs at the time of BCPA. BCPA can be performed with a low risk of morbidity and mortality in a wide range of patients. By using commonly acquired echocardiographic parameters, a low-risk subgroup of patients can be identified who can safely avoid preoperative cardiac catheterization.