"Single ventricle"

BACKGROUND: Previous studies on congenital heart diseases (CHD) associated with dextrocardia were based on selective patient databases and did not reflect the full spectrum of dextrocardia in the general population. Additionally, these studies had complex classification and presentation. Nor did these studies elaborate on the distribution of the associated CHD's complexity, the various segmental connections, and associated CHD among the four visceroatrial situs. METHODS: We retrospectively reviewed the medical records of 211 children with primary dextrocardia...

OBJECTIVE: Heterotaxy syndrome is a complex multisystem abnormality historically associated with high morbidity and mortality. We sought to evaluate the early and long-term outcomes after cardiac surgery in heterotaxy syndrome. METHODS: This is a single-center retrospective review of patients with heterotaxy syndrome undergoing single-ventricle palliation or primary or staged biventricular repair from 1998 to 2018. Patients were stratified by single ventricle versus biventricular physiology, and the severity of atrioventricular valve regurgitation...

OBJECTIVE: Pulmonary arterioplasty (PA plasty) at bidirectional cavopulmonary anastomosis (BDCA) is associated with increased morbidity, but outcomes to final stage palliation are unknown. We sought to determine the influence of PA plasty on pulmonary artery growth and hemodyamics at Fontan. METHODS: We retrospectively reviewed clinical data and outcomes for BDCA patients from 2006 to 2018. PA plasty was categorized by extent (type 1-4), as previously described...

OBJECTIVE: The best approach to minimize the observed higher mortality of newborn infants with low birth weight who require congenital heart surgery is unclear. This retrospective study was designed to review outcomes of newborn infants weighing <2000 g who have undergone cardiovascular surgery to identify patient parameters and clinical strategies for care associated with higher survival. METHODS: A retrospective chart review of 103 patients who underwent cardiovascular surgery from 2010 to 2021 who were identified as having low birth weight (≤2000 g)...

To evaluate the relationship of aortopulmonary collaterals and the development of central pulmonary arteries during staged palliation. A total of 287 patients, who underwent staged palliation with bidirectional cavopulmonary shunt and total cavopulmonary connection between 2008 and 2019, had available angiography. Pulmonary artery index was calculated using pulmonary angiography as described by Nakata and colleagues. Aortopulmonary collaterals were observed in 47 (16%) patients at stage II palliation, in 131 (46%) at total cavopulmonary connection, and afterwards in 49 (7%)...

OBJECTIVES: Symptomatic neonates and infants with Ebstein Anomaly (EA) require complex management. A group of experts was commissioned by the American Association for Thoracic Surgery to provide a framework on this topic focusing on risk stratification and management. METHODS: The EA Clinical Congenital Practice Standards Committee is a multinational and multidisciplinary group of surgeons and cardiologists with expertise in EA. A citation search in PubMed, Embase, Scopus, and Web of Science was performed using key words related to EA...

OBJECTIVES: Multicenter studies reporting outcomes following tracheostomy in children with congenital heart disease are limited, particularly in patients with single ventricle physiology. We aimed to describe clinical characteristics and outcomes in a multicenter cohort of patients with single ventricle physiology who underwent tracheostomy before Fontan operation. DESIGN: Multicenter retrospective cohort study.SETTING: Twenty-one tertiary care pediatric institutions participating in the Collaborative Research from the Pediatric Cardiac Intensive Care Society...

BACKGROUND: The Fontan operation is used to palliate single ventricle congenital heart defects (CHD) but poses significant morbidity and mortality risks. We present the design, planned analyses and rationale for a long-term Fontan cohort study aiming to examine the association of patient characteristics at the time of Fontan with post-Fontan morbidity and mortality. METHODS AND RESULTS: We used the Pediatric Cardiac Care Consortium (PCCC), a US-based, multicenter registry of pediatric cardiac surgeries to identify patients who underwent the Fontan procedure for single ventricle CHD between 1 and 21 years of age...

Improved survival rates for patients with a Fontan circulation has allowed more women with this complex cardiac physiology to contemplate pregnancy. However, pregnancy in women with a Fontan circulation is associated with a high risk of adverse maternal and fetal outcomes, high rates of miscarriage and preterm delivery. Factors associated with a successful pregnancy outcome are: younger age, normal body weight, absence of significant functional limitation, no Fontan-related complications, and well-functioning single ventricle physiology...

A heart with a borderline ventricle refers to a situation where there is uncertainty about whether the left or right underdeveloped ventricle can effectively support the systemic or pulmonary circulation with appropriate filling pressures and sufficient physiological reserve. Pediatric cardiologists often deal with congenital heart diseases (CHDs) associated with various degrees of hypoplasia of the left or right ventricles. To date, no specific guidelines exist, and surgical management may be extremely variable in different centers and sometimes even in the same center at different times...

Necrotizing enterocolitis (NEC) increases morbidity and mortality for infants with single ventricle heart disease (SVHD). While hematochezia often proceeds NEC not all hematochezia progresses to NEC. We aimed to examine the incidence, risk-factors, and outcomes associated with hematochezia and NEC for infants with SVHD. A single-center cohort study including SVHD patients requiring Stage I palliation from 12/2010 to 12/2015 was performed. Demographic, clinical, and outcome measures during the interstage period were abstracted from medical records...

The Fontan operation is the current standard of care for single-ventricle congenital heart disease. Individuals with a Fontan circulation (FC) exhibit central venous hypertension and face life-threatening complications of hepatic fibrosis, known as Fontan-associated liver disease (FALD). The fundamental biology and mechanisms of FALD are little understood. Here, we generated a transcriptomic and epigenomic atlas of human FALD at single-cell resolution using multiomic snRNA-ATAC-seq. We found profound cell type-specific transcriptomic and epigenomic changes in FC livers...

Patients with single ventricle continue to be a challenge for heart transplantation (HTx). A poor clinical condition, previous multiple surgical procedures, and possible allosensitization lead to an increased risk of complications. The results have improved in recent series (1). However, patients with preserved ventricular function continues to have a higher rate of mortality (2). Additionally, there are often anatomical alterations of the pulmonary arteries that complicate the surgery (1,3). This report presents a case of tricuspid atresia with pulmonary preassures that contraindicated Fontan surgery, with unfavorable anatomy of the central PAs and a poor clinical situation, in which an EXCOR® VAD (Berlin Heart) was used for subpulmonary mechanical circulatory support (MCS), associated with pulmonary bifurcation reconstruction, as a bridge to HTx...

Penn State University is developing a pediatric total artificial heart (pTAH) as a bridge-to-transplant device that supports infants and small children with single ventricle anomalies or biventricular heart failure to address high waitlist mortality rates for patients with severe congenital heart disease. Two issues with mechanical circulatory support devices are thrombus formation and thromboembolic events. This in vitro study characterizes flow within Penn State's pTAH under physiological operating conditions...

AIMS: The extracardiac conduit-Fontan (ECC) has become the preferred technique for univentricular heart palliation, but there are currently no data on the incidence of long-term arrhythmias. This study investigated the incidence of arrhythmias and relation to single ventricle morphology in the long-term follow-up in ECC. METHODS AND RESULTS: All patients with ECC performed in our Centre between 1987 and 2017 were included (minimum follow-up 5 years). Of 353 consecutive patients, 303 [57...

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OBJECTIVES: Fetuses with single ventricle physiology (SVP) exhibit reductions in fetal cerebral oxygenation with associated delays in fetal brain growth and neurodevelopmental outcomes. Maternal supplemental oxygen (MSO) has been proposed to improve fetal brain growth but current evidence on dosing, candidacy, and outcomes are limited. In this pilot study, we evaluated the safety and feasibility of continuous low-dose MSO in the setting of SVP. METHODS: This single-centre, open-label, pilot phase 1 safety and feasibility clinical trial included 25 pregnant individuals with a fetal diagnosis of SVP...

BACKGROUND: Pulmonary arteriovenous malformations (PAVMs) universally develop in patients with single ventricle congenital heart disease (CHD). Single ventricle PAVMs have been recognized for over 50 years, yet they are poorly understood, and we lack any medical therapies. To improve our understanding of single ventricle PAVM initiation and progression, we developed a surgical rat model of Glenn circulation and characterized PAVM physiology over multiple time points. METHODS: Using adult rats, we performed a left thoracotomy and end-to-end anastomosis of the left superior vena cava to the left pulmonary artery (unilateral Glenn), or sham surgical control...

OBJECTIVES: The aim of this study was to analyze the risk factors for acute events after systemic-to-pulmonary shunt (SPS) and to investigate the effectiveness of pulmonary blood flow regulation with a metal clip. METHODS: The case histories of 116 patients (78 biventricular [BV] and 38 single ventricle [SV] physiology) who underwent SPS between 2010 and 2021 were retrospectively reviewed. Our strategy was to delay SPS until 1 month of age; pulmonary blood flow (PBF) regulation by partial clipping of the graft, if needed...

Studies suggest the internal thoracic artery as a shunt option due to its growth potential. However, long-term data are lacking. Here, a patient with a failing single ventricle shunt had an enlarged internal thoracic artery. We followed the patient for 12 years after converting this artery into a Blalock-Taussig shunt, analysing its growth to assess its effectiveness.